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PMCID: PMC4481793 PMID: 26167022
Abstract
Lesions of the lower cranial nerves (LCN) are due to numerous causes, which need to be differentiated to optimize management and outcome. This review aims at summarizing and discussing diseases affecting LCN. Review of publications dealing with disorders of the LCN in humans. Affection of multiple LCN is much more frequent than the affection of a single LCN. LCN may be affected solely or together with more proximal cranial nerves, with central nervous system disease, or with nonneurological disorders. LCN lesions have to be suspected if there are typical symptoms or signs attributable to a LCN. Causes of LCN lesions can be classified as genetic, vascular, traumatic, iatrogenic, infectious, immunologic, metabolic, nutritional, degenerative, or neoplastic. Treatment of LCN lesions depends on the underlying cause. An effective treatment is available in the majority of the cases, but a prerequisite for complete recovery is the prompt and correct diagnosis.
LCN lesions need to be considered in case of disturbed speech, swallowing, coughing, deglutition, sensory functions, taste, or autonomic functions, neuralgic pain, dysphagia, head, pharyngeal, or neck pain, cardiac or gastrointestinal compromise, or weakness of the trapezius, sternocleidomastoid, or the tongue muscles. To correctly assess manifestations of LCN lesions, precise knowledge of the anatomy and physiology of the area is required.
요약
하부 뇌신경(LCN)의 병변은
여러 가지 원인에 의해 발생하며,
관리와 결과를 최적화하기 위해서는 원인을 구분해야 합니다.
이 리뷰는 LCN에 영향을 미치는 질병을 요약하고 논의하는 것을 목표로 합니다.
인간 LCN 장애를 다루는 출판물 리뷰.
다수의 LCN에 영향을 미치는 경우가
단일 LCN에 영향을 미치는 경우보다 훨씬 더 빈번합니다.
LCN은
단독으로 또는 더 가까운 두개골 신경, 중추신경계 질환, 비신경학적 장애와 함께
영향을 받을 수 있습니다.
LCN에 기인하는 전형적인 증상이나 징후가 있는 경우,
LCN 병변을 의심해야 합니다.
LCN 병변의 원인은
유전적, 혈관적, 외상적, 의원성, 감염성, 면역학적, 대사적, 영양적, 퇴행성, 또는
종양성으로 분류할 수 있습니다.
LCN 병변의 치료는
근본적인 원인에 따라 달라집니다.
대부분의 경우 효과적인 치료가 가능하지만,
완전한 회복을 위해서는 신속하고 정확한 진단이 전제 조건입니다.
언어 장애, 삼킴, 기침, 연하, 감각 기능, 미각, 자율 기능,
신경통, 연하 곤란, 머리, 인두, 목 통증, 심장 또는 위장 장애, 또는 승모근, 흉쇄유돌근, 혀 근육의 약화 등의
증상이 있는 경우, LCN 병변을 고려해야 합니다.
disturbed speech,
swallowing,
coughing,
deglutition,
sensory functions,
taste, or
autonomic functions,
neuralgic pain,
dysphagia,
head, pharyngeal, or neck pain,
cardiac or gastrointestinal compromise, or
weakness of the trapezius, sternocleidomastoid, or the tongue muscles
Idiopathic spinal accessory nerve palsy
A case report and review of the literature
Gürsoy, Azize Esra; Babacan-Yildiz, Gülsen; Kolukisa, Mehmet; Celebi, Arif
Neurology India 61(1):p 82-83, Jan–Feb 2013. | DOI: 10.4103/0028-3886.108021
Metrics
Sir,
Spinal accessory nerve (SAN) palsy is an uncommon pure motor mononeuropathy. Fibers of the spinal branch of the SAN innervate the sternocleidomastoid and trapezius muscles.[1] We present a case with SAN palsy.
A 34-year-old man presented with the complaint of asymmetry of the shoulders since 9 months. He denied any neck or shoulder pain and could not recall a specific precipitating traumatic event. On neurologic examination, he had an asymmetric neckline and drooping of left shoulder. He was able to abduct his arms beyond the horizontal position and actively complete a full range of motion. He had mild weakness of the left trapezius muscle with no wasting or weakness of the ipsilateral sternocleidomastoid muscle. Magnetic resonance imaging (MRI) revealed atrophy of the left trapezius muscle [Figure 1]. Nerve conduction study (NCS) of the left spinal accessory nerve, with surface stimulation along the posterior border of the sternocleidomastoid muscle and recording from the trapezius, recorded compound muscle action potential with lower amplitude compared to the right side (4.2 mV vs. 6.6 mV). Rest of the NCS was normal. Needle examination of left upper trapezius muscle showed intense positive sharp waves and fibrillation potentials and moderatly reduced recruitment of motor unit potentials, with prolonged duration and slightly increased amplituded. Rest of the needle examination was normal. These findings were consistent with SAN palsy distal to the innervation site of the sternocleidomastoid muscle. Consequently, a diagnosis of chronic left idiopathic SAN palsy was considered and he was prescribed progressive strengthening exercise of the trapezius.
척추 부신경(SAN) 마비는
흔하지 않은 순수 운동성 단일 신경병증입니다.
척추부속신경의 척추 가지의 섬유는
흉쇄유돌근과 승모근을 자극합니다.[1]
우리는 척추부속신경 마비 사례를 소개합니다.
34세 남성이 9개월 전부터 어깨의 비대칭에 대한 불만을 호소했습니다.
그는 목이나 어깨 통증을 부인했으며, 특정 외상성 사건을 기억하지 못했습니다.
신경학적 검사 결과, 그는 목의 비대칭과 왼쪽 어깨의 처짐이 있었습니다.
그는 팔을 수평 위치 이상으로 외전할 수 있었고, 능동적으로 전체 동작 범위를 완료할 수 있었습니다.
그는 왼쪽 승모근의 약한 약화 증세를 보였지만, 동측 흉쇄유돌근의 위축이나 약화는 없었습니다.
자기공명영상(MRI) 검사 결과, 왼쪽 승모근의 위축이 나타났습니다[그림 1]. 흉쇄유돌근의 뒤쪽 경계를 따라 표면 자극을 가하고 승모근에서 기록한 왼쪽 척추 보조 신경의 신경 전도 연구(NCS)에서, 오른쪽(4.2mV 대 6.6mV)에 비해 진폭이 낮은 복합 근육 활동 전위를 기록했습니다.
나머지 NCS는 정상입니다.
왼쪽 상부 승모근의 바늘 검사는 강렬한 양의 날카로운 파동과 세동 가능성을 보였으며,
운동 단위 잠재력의 모집은 약간 감소하고 지속 시간은 길어졌으며 진폭은 약간 증가했습니다.
바늘로 찔러서 검사한 나머지 부위는 정상입니다.
이 결과는 흉쇄유돌근의 신경 분포 부위에서 원위부까지 SAN 마비가 일어난다는 사실과 일치합니다. 따라서 만성 왼쪽 특발성 SAN 마비 진단이 고려되었고, 그는 승모근의 점진적 강화 운동을 처방받았습니다.
Figure 1:
Magnetic resonance imaging of the neck showing muscle atrophy of the left trapezius muscle (arrow)
The most common cause of SAN palsy is iatrogenic damage during neck surgery.[2] The superficial location of the SAN in the posterior cervical triangle makes it very susceptible to injury. Some sports activities and deep tissue massage may be responsible for spinal accessory nerve palsy in exceptional cases.[2] The diagnostic evaluation of SAN palsy should be based on history and physical findings and should be confirmed by NCS and electromyogarphy. SAN palsy may lead to dysfunction of the trapezius muscle, which results in drooping of the shoulder, asymmetry of the neckline, downward rotation and protraction of the scapula, and weakness of forward elevation and abduction movements, but there are many partial forms that are well-tolerated.[1] Although MRI reveals muscle abnormalities in more than 50% of cases, the most helpful diagnostic tests in confirming SAN palsy are nerve conduction studies and electromyography.[3] The role of EMG in predicting prognosis is limited. Good outcomes were reported in some cases of SAN palsy, even though dense fibrillation potentials were observed 9.5 months after onset.[4] In conclusion, spontaneous SAN palsy of unknown origin is a very rare neuropathy. Clinical, electrophysiological, and MRI examinations should be done appropriately for the accurate diagnosis of SAN palsy in patients with an asymmetric neckline.
목의 자기공명영상에서 왼쪽 승모근(화살표)의 근육 위축이 보입니다.
척추부신경마비의 가장 흔한 원인은
목 수술 중 의인성 손상에 의한 것입니다.[2]
척추부신경은 후방 경추 삼각형에 위치해 있기 때문에 부상에 매우 취약합니다.
일부 스포츠 활동과 심부 조직 마사지는
예외적인 경우 척추부신경마비의 원인이 될 수 있습니다.[2]
척추부신경마비의 진단 평가는
병력 및 신체적 소견에 근거해야 하며,
근전도 검사 및 근전도 검사로 확인해야 합니다.
삼두근 마비는 승모근의 기능 장애를 유발할 수 있으며, 이로 인해 어깨가 처지고, 목선이 비대칭이 되며, 견갑골이 아래로 회전하고 뻗어나가는 현상이 발생하고, 앞으로 들어 올리거나 옆으로 벌리는 동작이 약해질 수 있습니다. 그러나 부분적인 형태가 많습니다. 잘 견디는[1] 것입니다. MRI는 50% 이상의 사례에서 근육 이상을 보여 주지만, SAN 마비를 확인하는 데 가장 도움이 되는 진단 검사는 신경 전도 검사 및 근전도 검사입니다[3]. 예후를 예측하는 데 있어 근전도 검사의 역할은 제한적입니다. 발병 후 9.5개월이 지난 후에도 심방세동 가능성이 관찰되었음에도 불구하고, 일부 SAN 마비의 경우 좋은 결과가 보고되었습니다.[4] 결론적으로, 원인을 알 수 없는 자발적 SAN 마비는 매우 드문 신경병증입니다. 임상, 전기생리학, MRI 검사를 적절히 실시하여 비대칭 목선을 가진 환자의 SAN 마비를 정확하게 진단해야 합니다.
LCN 병변의 증상을 정확하게 평가하려면
해당 부위의 해부학과 생리학에 대한 정확한 지식이 필요합니다.
Keywords: Accessory, cranial nerves, glossopharyngeal, hypoglossal, infection, neoplasm, trauma, vagus, vascular
Introduction
Lower cranial nerves (LCN) include the paired 9th (glossopharyngeal), 10th (vagal), 11th (accessory) and 12th (hypoglossal) cranial nerves.[1] Particularly the 9th, 10th, and 12th cranial nerves are involved in the execution of crucial physiological functions, such as swallowing, tasting, speech, heart rate and blood pressure control, and peristalsis. In addition to motor fibers, LCN carry sensory fibers and autonomic fibers. LCN are affected in a number of pathologic conditions. This review aims at summarizing and discussing diseases associated with LCN, its diagnosis, treatment, and outcome. Due to limitations in space, synaptic and postsynaptic neuromuscular transmission disease and disorders of the skeletal muscles supplied by LCN were excluded, and the discussion about treatment and outcome limited.
소개
하부뇌신경(LCN)은 쌍으로 존재하는
9번(설인두신경),
10번(미주신경),
11번(부신경),
12번(설하신경)을 포함합니다.1]
9th (glossopharyngeal),
10th (vagal),
11th (accessory) and
12th (hypoglossal) cranial nerves
특히 9번, 10번, 12번 뇌신경은
삼킴, 미각, 언어, 심박수 및 혈압 조절, 연동 운동과 같은
중요한 생리학적 기능을 수행하는 데 관여합니다.
운동 신경 섬유 외에도
LCN은 감각 신경 섬유와 자율 신경 섬유를 전달합니다.
LCN은 여러 병리학적 조건에 영향을 받습니다.
이 리뷰는 LCN과 관련된 질병, 진단, 치료, 그리고
결과를 요약하고 논의하는 것을 목표로 합니다.
공간 제약으로 인해 시냅스 및 시냅스 후 신경근 전달 질환과 LCN에 의해 공급되는 골격근의 장애는 제외되었으며, 치료 및 결과에 대한 논의는 제한적입니다.
Methods
Data for this review were identified by searches of MEDLINE, Current Contents, PubMed, and references from relevant articles using the search terms “glossopharyngeal nerve,” “vagal nerve,” “accessory nerve,” “hypoglossal nerve,” and “cranial nerves,” in combination with “genetic,” “hereditary,” “motor neuron disease,” “stroke,” “vascular,” “vasculitis,” “trauma,” “head injury,” “iatrogenic,” “infections,” “immunologic,” “immune neuropathies,” “multiple sclerosis,” “diabetes,” “vitamin deficiency,” “degeneration,” “malformation,” “neoplasm,” and “carcinoma.” Randomized (blinded or open-label) clinical trials, longitudinal studies, case series, and case reports were considered. Abstracts and reports from meetings were not included. Only articles published in English between 1966 and 2014 were considered. Appropriate papers were studied and discussed for their suitability to be incorporated in this review.
Anatomy and Physiology of Lower Cranial Nerves
To understand the complicated interplay between the four LCN and their anatomy and various functions, it is essential to realize the basics of anatomy and physiology.
하부 뇌신경의 해부학과 생리학
네 개의 LCN과 그 해부학적 구조, 그리고 다양한 기능 사이의 복잡한 상호 작용을 이해하기 위해서는 해부학과 생리학의 기초를 이해하는 것이 필수적입니다.
9th cranial (glossopharyngeal) nerve
The ninth cranial nerve is a mixed nerve carrying motor efferents and sensory afferents. The nerve mediates five distinct functions: A branchial motor function providing voluntary control of the stylopharyngeal muscle (dilates and elevates pharynx during swallowing and speech); visceral motor functions to innervate the parotid gland via preganglionic fibers to the otic ganglion and postganglionic fibers to the parotid gland; visceral sensory functions providing visceral sensory input from the carotid sinus and carotid bodies, general somatic afferent functions providing sensory information for the skin of the external ear, the internal surface of the tympanic membrane, the upper pharynx, the soft palate, the tonsils, and the posterior third of the tongue; and a special sensory function providing taste sensation from the posterior third of the tongue.[2] Branches of the nerve include the tympanic, the pharyngeal, the lingual, the sinus caroticus, the tonsillar, and the stylopharyngeal branch. There is also a communicating branch to the vagal nerve.[2]
The branchial motor branch originates from the nucleus ambiguus in the reticular formation of the medulla, emerges from the lateral aspect of the medulla, exits the skull via the jugular foramen, and descends to the styloid process to innervate the target muscles.[3] The visceral (parasympathetic) motor branch of the nerve innervates the ipsilateral parotid gland. Preganglionic fibers originate from the inferior salivatory nucleus (receives afferents from the hypothalamus and the olfactory system) to exit the medulla between olive and inferior cerebellar peduncle. Together with other components of the nerve, parasympathetic fibers cross the two glossopharyngeal ganglia in the jugular foramen to form the tympanic nerve. The tympanic nerve ascends into the tympanic cavity to form a plexus, which provides the general sensation of the middle ear. Parasympathetic fibers leave the plexus as the lesser petrosal nerve, re-enter the middle cranial fossa to exit the skull via the foramen ovale along with the mandibular nerve and synapses in the otic ganglion. Postganglionic fibers travel with the auriculo-temporal branch of the mandibular nerve to enter the parotid gland. The visceral sensory component carries impulses from the baroreceptors of the carotid sinus and the chemoreceptors of the carotid body via the sinus nerve to join the 9th cranial nerve at the inferior glossopharyngeal ganglion. From there these projections reach the tractus solitarius to synapse in the caudal nucleus solitarius. Connections are made with the reticular formation and the hypothalamus to mediate cardiovascular and respiratory reflex responses to changes of blood pressure or CO2/O2. The general sensory component originates from the skin of the external ear, the upper pharynx, and the posterior third of the tongue to travel via the pharyngeal branch and the tympanic nerve to the spinal trigeminal tract. Ascending neurons project to the thalamus. These sensory fibers mediate the afferent limb of the gag reflex, while the efferent impulse is carried by the branchial motor fibers of the vagal nerve. The 9th cranial nerve is also involved in the afferent side of the cough reflex.[4] Special sensory fibers carry taste sensation from the posterior third of the tongue to the inferior ganglion, and then pass the jugular foramen to ascend to the tractus solitarius and synapse with the nucleus solitarius.[1,2] Hiccups are due to a brief contraction of the inspiratory muscles plus a glottis adduction of the stylopharyngeal muscles.[4]
9번 뇌신경(설인두신경)
9번 뇌신경은
운동 원심성과 감각 구심성을 전달하는 혼합 신경입니다.
이 신경은 다섯 가지 뚜렷한 기능을 중재합니다.
인두근을 자발적으로 제어하는 구인두 운동 기능(삼키거나 말할 때 인두를 확장하고 들어 올림);
귀밑샘을 자극하는 내장 운동 기능(이전에 신경절 섬유로 귀 신경절에 전달되고, 이후에 신경절 섬유로 귀밑샘에 전달됨);
경동맥동과 경동맥체로부터 내장 감각 입력을 제공하는 내장 감각 기능;
구강과 인두의 구심성 감각 입력을 제공하는 일반적인 체성 구심성 기능;
외이의 피부, 고막의 내면, 상부 인두, 입천장, 편도, 혀의 뒤쪽 1/3; 그리고 혀의 뒤쪽 1/3에서 미각을 제공하는 특별한 감각 기능.
신경의 가지에는
고막, 인두, 설측, 경동맥동, 편도, 인두두 가지가 포함됩니다.
미주신경으로 통하는 분지 신경도 있습니다.[2]
분지 운동 분지는
수질의 망상 조직에 있는 모호핵에서 시작되어 수질의 측면을 통해 나오고,
경정맥을 통해 두개골을 빠져나가,
스타일러스 과정을 따라 내려가 대상 근육을 자극합니다.[3]
신경의 내장(부교감) 운동 분지는
동측 이하선에 자극을 줍니다.
구신경 섬유는
시상하부와 후각계로부터 구심성 신경을 받는 하부 타액선핵에서 시작되어,
올리브와 하부 소뇌돌기 사이의 수질에서 빠져나옵니다. 신
경의 다른 구성 요소와 함께 부교감 신경 섬유는
경정공의 두 개의 설인두 신경절 사이를 가로질러 고막 신경을 형성합니다.
고막 신경은
고막강으로 올라가서 신경총을 형성하는데,
이 신경총은 중이의 전반적인 감각을 제공합니다.
부교감 신경 섬유는
작은 후두골 신경으로 신경총을 떠나서 두개골 안으로 다시 들어가서,
하악 신경 및 달팽이관 신경절의 시냅스와 함께 난소공을 통해 두개골 밖으로 나옵니다.
신경절 후 섬유는
하악 신경의 귀측 측두 가지와 함께 귀밑샘으로 들어갑니다.
내장 감각 구성 요소는
부비동 신경을 통해 경동맥 부비동의 압력 수용체와 경동맥체의 화학 수용체로부터 자극을 전달받아
하악 인두 신경절에서 제9뇌신경에 합류합니다.
그곳에서 이 돌기는 꼬리 솔리타리우스 핵의 솔리타리우스 시냅스로 이어집니다. 망상형성과 시상하부와 연결되어 혈압이나 CO2/O2의 변화에 대한 심혈관 및 호흡기 반사 반응을 조절합니다. 일반적인 감각 구성 요소는 외이, 상부 인두, 혀의 뒤쪽 1/3의 피부에서 시작되어 인두 가지와 고막 신경을 통해 척추 삼차 신경으로 이동합니다. 상승하는 뉴런은 시상까지 연결됩니다. 이러한 감각 섬유는 구역반사의 구심성 사지를 매개하는 반면, 원심성 자극은 미주신경의 분지 운동 섬유에 의해 전달됩니다. 제9뇌신경은 기침 반사의 구심성 측면에도 관여합니다. [4] 특수 감각 섬유는 혀의 뒤쪽 1/3에서 미각을 하부 신경절로 전달한 다음, 경정맥을 통과하여 상승합니다. 단일핵과 시냅스[1,2] 딸꾹질은 흡기근의 짧은 수축과 인두두근의 성문 내전으로 인해 발생합니다.[4]
10th cranial (vagal) nerve
The vagal nerve is a mixed nerve carrying motor efferents and sensory afferents. The nerve conducts five distinct qualities, which are carried along general visceral efferent fibers (parasympathetic innervation of pharyngeal, laryngeal, bronchial, and gastrointestinal mucosa), general visceral afferent fibers (sensory information from the thoracic and abdominal viscera, the aortic body, aortic arch), special visceral afferent fibers (carry taste of the epiglottal region), general somatic afferent fibers (carry sensation from the external auditory meatus, outer tympanic membrane, back of the ear, part of meninges, pharynx), and along special visceral efferent fibers, which innervate skeletal muscles of the pharynx and larynx. Skeletal muscles innervated by the vagal nerve include the cricothyroid, levator veli palatini, salpingopharyngeus, palatoglossal, palatopharyngeus pharyngeal constrictor, and the laryngeal muscles (except cricothyroid). Innervation of these muscles is involved during speech or opening of the larynx during breathing. Efferent parasympathetic fibers control heart rate, peristalsis, and sweating. Stimulation of the efferent parasympathetic fibers lowers heart rate or blood pressure. 80–90% of the vagal fibers are afferent, only 10–20% are efferent fibers.[5]
Vagal nerve fibers originate from or converge to four medullar nuclei, which include the dorsal nucleus of the vagal nerve (responsible for parasympathetic output to viscera), nucleus ambiguus (origin of branchial efferent motor fibers and of preganglionic parasympathetic to the heart), solitary nucleus (receives taste information and afferents from viscera), and the spinal trigeminal nucleus (receives sensory input from the outer ear, dura, posterior cranial fossa, and mucosa). The vagal nerve leaves the medulla between the pyramid and inferior cerebellar peduncle, crosses the jugular foramen, and passes into the carotid sheath down to the neck, chest and abdomen. It splits into the auricular, pharyngeal (innervates palate and pharynx), superior laryngeal (innervates constrictor and cricothyroid), and recurrent laryngeal nerves, the anterior and posterior vagal trunk, and provides superior and inferior cervical cardiac branches and thoracic cardiac branches to the heart and the esophageal and pulmonary plexus (vagus nerve branches). The superior laryngeal nerve divides into the internal and external laryngeal nerves. The external laryngeal branch supplies the inferior constrictors. The vocal cord is also innervated by the superior laryngeal nerve and the external and internal rami of the inferior laryngeal nerve.[5]
The right vagus crosses anteriorly of the subclavian artery, runs posterior of the superior vena cava, descends posterior to the right main bronchus to form the cardiac, pulmonary and esophageal plexus. Distally, the right vagal nerve passes over into the posterior vagal trunk, which crosses the diaphragm through the esophageal hiatus. The right recurrent laryngeal nerve deviates from the right vagal nerve and hooks around the right subclavian artery and ascends to the neck. From the left vagal nerve deviates the left recurrent laryngeal nerve, which hooks around the aortic arch to ascend back to the neck. After having contributed to the esophageal, cardiac, and pulmonary plexus, the left vagal nerve forms the anterior vagal trunk, which enters the abdomen via the esophageal hiatus. The right vagal nerve innervates the sinusnode, whereas the left vagal nerve innervates the atrioventricular-node. One of the most important reflexes mediated by the vagal nerve is the gag reflex. Also, the cough reflex is partially mediated via general visceral afferent parasympathetic fibers.[4] The cough reflex is initiated by cough receptors. Vagal sensory fibers together with trigeminal and glossopharyngeal fibers carry information from the cough receptors to the cerebral cough center (nucleus tractus solitarius). From there efferent fibers of the phrenic nerve, spinal nerves, and laryngeus recurrens nerve project to the diaphragm, abdominal, intercostal, and laryngeal muscles.[4] Parasympathetic compensation of sympathetic overstimulation in response to pain, carotid sinus massage, the Valsalva manoeuvre, or gastrointestinal illness, may cause a vasovagal syncope.
제10뇌신경(미주신경)
미주신경은
운동 원심성(10%)과 감각 구심성(90%)을 전달하는 혼합 신경입니다.
이 신경은 다섯 가지 특성을 전달하는데, 이 특성은
일반 내장 원심성 섬유(인두, 후두, 기관지, 위장 점막의 부교감 신경 분포),
일반 내장 구심성 섬유(흉부 및 복부 내장, 대동맥 몸체, 대동맥 궁에서 오는 감각 정보),
특수 내장 구심성 섬유( 후두개 부위의 맛을 전달하는 구심성 섬유,
일반적인 체성 구심성 섬유(외이도, 외이막, 귀 뒤쪽, 수막의 일부, 인두에서 오는 감각을 전달), 그리고
인두와 후두의 골격근을 자극하는 특수 내장 원심성 섬유를 따라 이어집니다.
미주신경에 의해 자극되는 골격근에는
갑상갑상근, 갑상설골근, 설인두근, 설측설골근, 설인두인두수축근, 그리고 후두근(갑상갑상근 제외)이 포함됩니다.
이 근육의 신경 분포는
말하기 또는 호흡 중 후두 개방과 관련이 있습니다.
원심성 부교감 신경 섬유는
심박수, 연동 운동, 발한을 조절합니다.
원심성 부교감 신경 섬유의 자극은
심박수 또는 혈압을 낮춥니다.
미주 신경 섬유의 80-90%는 구심성이고,
10-20%만이 원심성입니다.5]
미주신경 섬유는
4개의 수질핵에서 시작되거나 수렴하는데,
여기에는 미주신경의 등쪽 핵(내장 부교감 신경 분포를 담당),
모호핵(원심성 운동 섬유와 심장 부교감 신경절(심장 부교감 신경),
독방핵(내장으로부터 미각 정보와 구심성 신호를 받음),
척추 삼차신경핵(외이, 경막, 후두개, 점막으로부터 감각 입력을 받음).
미주신경은 피라미드와 소뇌하돌기 사이의 수질에서 빠져나와 경정맥을 가로질러 경동맥으로 들어가 목, 가슴, 복부로 내려갑니다. 이 신경은 귀신경, 인두신경(구개와 인두를 자극), 상후두신경(성대신경과 갑상갑상연골을 자극), 그리고 재발성 후두신경, 전방 및 후방 미주신경줄기로 나뉘며, 상부 및 하부 경추심장 가지와 흉부심장 가지, 그리고 심장 및 식도 및 폐 신경총(미주신경 가지)을 제공합니다. 상후두신경은 내후두신경과 외후두신경으로 나뉩니다. 외부 후두 신경은 하후두근을 공급합니다. 성대는 또한 상후두 신경과 하후두 신경의 외부 및 내부 가지에 의해 자극을 받습니다.5]
오른쪽 미주신경은 쇄골하 동맥의 앞쪽을 가로질러 상대정맥의 뒤쪽을 지나 오른쪽 주기관지의 뒤쪽으로 내려가서 심장, 폐, 식도 신경총을 형성합니다. 원위부에서는 오른쪽 미주신경이 후방 미주신경줄기로 넘어가서 식도열공을 통해 횡격막을 가로지릅니다. 오른쪽 후두신경은 오른쪽 미주신경에서 벗어나 오른쪽 쇄골하동맥을 감싸고 목으로 올라갑니다. 왼쪽 미주신경에서 왼쪽 후두신경이 갈라져서 대동맥궁을 감싸고 목으로 올라갑니다. 식도, 심장, 폐 신경총에 기여한 후, 왼쪽 미주신경은 앞쪽 미주신경줄기를 형성하고, 이 신경줄기는 식도열공을 통해 복부로 들어갑니다. 오른쪽 미주신경은 부비동결절에 분포하고, 왼쪽 미주신경은 방실결절에 분포합니다. 미주신경이 매개하는 가장 중요한 반사작용 중 하나는 구역반사입니다. 또한, 기침 반사는 부분적으로 내장 구심성 부교감 신경 섬유에 의해 매개됩니다. [4] 기침 반사는 기침 수용체에 의해 시작됩니다. 미주 감각 섬유는 삼차신경 및 설인두신경 섬유와 함께 기침 수용체에서 뇌의 기침 중심(솔리타리우스핵)으로 정보를 전달합니다. 거기에서, 횡격막, 복부, 늑간, 후두 근육으로의 신경섬유가 분포되어 있습니다.4] 통증, 경동맥 마사지, 발살바 기동, 또는 위장병에 대한 교감신경의 과다 자극에 대한 부교감신경의 보상 작용은 혈관미주신경성 실신을 유발할 수 있습니다.
11th cranial (accessory) nerve
The accessory nerve is anatomically split into a spinal and a cranial portion. Fibers of the spinal portion originate from the upper spinal cord (spinal accessory nucleus located in the postero-lateral aspect of the anterior horn). The cranial portion originates from the nucleus ambiguus, which is continuous with the lateral horn of the cervical segments. The spinal roots form the spinal accessory component, which enters the skull through the foramen magnum. The cranial part descends from the medulla oblongata to connect with the spinal accessory component. The nerve then courses along the skull to the jugular foramen through which it exits together with the 9th and 10th cranial nerve. The 10th cranial nerve lies most medial in the foramen. The 9th cranial nerve exits together with the inferior petrosal sinus through the anterior compartment of the foramen. Vagus and accessory nerves exit via the middle compartment of the foramen. The sigmoid sinus exits via the posterior compartment of the foramen. Shortly before crossing the foramen, the cranial component branches off the accessory nerve to join the vagal nerve (the 11th cranial nerve is a transitional nerve). In the neck, the nerve crosses the internal jugular vein anteriorly (80% of cases) or posteriorly (20% of cases) to enter the target muscles shortly afterwards. Due to this unique anatomic course, the accessory nerve is the only cranial nerve which enters and exits the skull and is thus sometimes not regarded as a true cranial nerve.[6] Physiologically, the accessory nerve is a mixed or transitional nerve conducting general somatic qualities (innervation of the sternocleidomastoid and trapezius muscles) originating from the cranial portion and a distinct modality originating from the spinal portion.[7] Anastomoses between the spinal component of the accessory nerve and the dorsal roots of the cervical nerves exist below level C1.[8,9]
제11뇌신경(부신경)
부신경은
해부학적으로
척추 부분과 두개골 부분으로 나뉩니다.
척추 부분의 섬유는
상부 척수(전방 경적의 후측면에 위치한 척추부 신경핵)에서 시작됩니다.
두개골 부분은 자궁 경부의 측면 경적과 연속적인 모호핵에서 시작됩니다. 척추 신경근은 척추 보조 구성 요소를 형성하며, 이 구성 요소는 대공을 통해 두개골 안으로 들어갑니다. 두개골 부분은 연수에서 내려와 척추 보조 구성 요소와 연결됩니다. 그런 다음 신경은 두개골을 따라 경정 공으로 이동하여 9번과 10번 뇌신경과 함께 빠져나갑니다. 10번 뇌신경은 대공의 가장 안쪽에 위치합니다. 9번 뇌신경은 하악골동과 함께 전방 구획을 통해 두개골의 앞쪽을 통해 빠져나갑니다.
미주신경과 부신경은 두개골의 중간 구획을 통해 빠져나갑니다. S자동맥은 두개골의 뒤쪽 구획을 통해 빠져나갑니다. 두개골의 앞쪽 부분이 두개골의 앞쪽을 통과하기 직전에 부신경에서 갈라져 나와 미주신경에 합류합니다(11번 뇌신경은 과도기적 신경입니다). 목에서, 신경은 내부 경정맥을 앞쪽(80%의 경우) 또는 뒤쪽(20%의 경우)으로 지나가면서 곧바로 목표 근육으로 들어갑니다.
이러한 독특한 해부학적 경로 때문에,
부신경은 두개골 안팎으로 들어오고 나가는 유일한 뇌신경이기 때문에
때로는 진정한 뇌신경으로 간주되지 않습니다.
생리학적으로,
부신경은 일반적인 체성질(흉골의 신경 분포)을 수행하는 혼합 신경 또는 과도 신경입니다.
두개골 부분에서 시작되는 쇄골하근과 승모근, 그리고
척추 부분에서 시작되는 뚜렷한 양식.[7]
보조 신경의 척추 부분과 경추 신경의 등쪽 뿌리 사이의 문합은
12th cranial (hypoglossal) nerve
The hypoglossal nerve innervates the muscles of the tongue and is involved in tongue movements during speech, food manipulation, and swallowing.[1] The nerve is pure motor.[10] The nerve directs not only voluntary activities of the tongue, but also involuntary functions, such as clearing the mouth of saliva by swallowing. The nerve is also involved in adjusting the tongue to new movements to create desired sounds when learning a new language.[10] The nerve originates from the hypoglossal nucleus.[1] It leaves the medulla oblongata between the olive and the pyramid to pass through the hypoglossal canal. Immediately after leaving the skull it gives off a meningeal branch and unites with a branch of the anterior root C1, which innervates the geniothyroid and thyrohyoid muscles. The nerve then winds behind the 10th cranial nerve and passes between internal carotid artery and internal jugular vein to the posterior belly of the digastric muscle to continue to the submandibular region lateral to the hypoglossal muscle and inferior of the lingual nerve to finally reach the tongue. All muscles of the tongue are supplied except for the palatoglossal muscle, which is innervated by the 9th cranial nerve. There are anastomoses between the hypoglossal nerve and other caudal cranial nerves and the cervical plexus.
제12뇌신경(설하신경)
설하신경은
혀의 근육을 자극하고, 말하기, 음식 다루기, 삼키기 등의 과정에서
혀의 움직임에 관여합니다.
이 신경은 순수한 운동 신경입니다.
이 신경은 혀의 자발적인 활동뿐만 아니라
삼킴을 통해 입안의 침을 뱉는 것과 같은 비자발적인 기능도 지시합니다.
신경은 또한
새로운 언어를 배울 때 원하는 소리를 내기 위해 혀를 새로운 움직임에 맞추는 데 관여합니다.10]
신경은
설하핵에서 시작됩니다.1]
신경은 올리브와 피라미드 사이의 수질에서 출발하여 설하관을 통과합니다.
두개골을 떠난 직후, 그것은 경막 가지(meningeal branch)를 내보내고, 갑상선 및 갑상선하근을 자극하는 앞쪽 C1근의 가지와 합쳐집니다. 신경은 10번 뇌신경 뒤로 감겨 들어가서 내부 경동맥과 내부 경정맥 사이를 지나서 늑간근의 뒤쪽 복부로 들어가서 설하근의 옆쪽과 설하신경의 아래쪽으로 계속해서 내려가서 결국 혀에 도달합니다. 혀의 모든 근육은 9번 뇌신경에 의해 자극을 받는 구개설측근을 제외한 모든 근육에 공급됩니다. 설측신경과 다른 꼬리쪽 뇌신경, 경추 신경총 사이에는 문합이 있습니다.
Classification of Lower Cranial Nerve Lesions
Lesions of the LCN may be classified according to various aspects. According to the number of nerves affected they may be categorized as either single nerve lesions or as multiple nerve lesions. According to the impaired function, they may be qualified as motor (muscle or visceral) or sensory (visceral, general somatic, special). LCN lesions may be also classified whether the nerves are involved individually or whether other cranial nerves, the brain, the spinal cord or the spinal nerves are additionally affected. Another categorization is based on the cause of the lesion, which may be genetic, vascular, traumatic, iatrogenic, infectious, immunologic, metabolic, nutritional, degenerative, or neoplastic.
하두개신경 병변의 분류
하두개신경 병변은 다양한 측면에 따라 분류될 수 있습니다.
영향을 받는 신경의 수에 따라
단일 신경 병변 또는
다중 신경 병변으로 분류될 수 있습니다.
손상된 기능에 따라 운동(근육 또는 내장) 또는 감각(내장, 일반 체성, 특수)으로 분류될 수 있습니다.
LCN 병변은 신경이 개별적으로 관련되어 있는지 또는
다른 뇌신경, 뇌, 척수 또는 척수신경이 추가로 영향을 받는지에 따라 분류될 수 있습니다.
또 다른 분류는 병변의 원인에 따라 이루어지며,
원인은 유전적, 혈관적, 외상적, 의원성, 감염성, 면역성, 대사성, 영양성, 퇴행성 또는 종양성일 수 있습니다.
Symptoms of Lower Cranial Nerve Lesions
Symptoms arising from lesions of LCN correlate with the various functions of the four nerves. Thus, symptoms of LCN lesions include dysphagia, dysarthria, hoarse voice, chocking during ingestion of fluids, taste disturbances (posterior third of tongue), sensory disturbances (posterior third of the tongue, soft palate, throat, external auditory meatus), palpitations, tachycardia, bradycardia, tachypnea, bradypnea, dyspnea, arterial hypotension, arterial hypertension, fainting, syncope, dizziness, pain in the posterior oral cavity, the face, the neck, the head, obstipation, or diarrhoea. Affection of the 11th cranial nerve may lead to weakness of head rotation, head anteflexion, or shoulder elevation, to scapular winging or to shoulder pain. Affection of the 12th cranial nerve may lead to slurring of speech and the feeling of a thick, heavy, or clumsy tongue. If additional neural or nonneural structures are involved, symptomatology may be even broader.
Affection of the LCN may go along with pain, such as neuralgias, dysesthesias, paresthesias, muscle pain, or pain with swallowing. Neuralgias are characterized by paroxysmal painful attacks of electric shock-like sensation, occurring spontaneously or evoked by innocuous stimuli in specific trigger areas.[11] Pain is usually experienced in the distribution of one or several nerves. Neuralgias of the LCN are the rare glossopharyngeal neuralgia and the even more rare neuralgia of the laryngeal nerve. Glossopharyngeal neuralgia is characterized by excruciating pain attacks of the short duration of the ear, pharynx, neck, tonsil, or base of the tongue. Pain may be triggered by contact of the tongue with the teeth[12] or with the back of the tongue.[13] Glossopharyngeal neuralgia may be associated with syncope (fainting) in 10% of the cases due to reflex bradycardia (efferent limb of reflex via vagal nerve) or occasionally induced by swallowing. Glossopharyngeal neuralgia is most frequently primary (compression of the nerve by adjacent vessel loop)[14] or secondary to an intrinsic brain lesion such as in multiple sclerosis[13,15] or in the Eagle-syndrome.[16] Neuralgia of the laryngeal nerve is a rare condition and may be due to a deviated hyoid bone,[17] due to previous acute laryngitis,[18] a pharyngeal diverticulum,[19] or idiopathic.[20] Patients present with paroxysmal, lancing pain located to the area of the thyrohyoid membrane, which can be induced or worsened by swallowing.[17]
하부뇌신경 병변의 증상
하두신경 병변으로 인해 발생하는 증상은
네 가지 신경의 다양한 기능과 관련이 있습니다.
따라서,
하두신경 병변의 증상으로는
연하곤란,
구음장애,
쉰 목소리,
액체 섭취 시 질식,
미각 장애(혀의 뒤쪽 1/3),
감각 장애(혀의 뒤쪽 1/3, 입천장, 목, 외이도),
심계항진,
빈맥 심장박동수 증가,
심박수 감소,
빈호흡,
저호흡,
호흡곤란, 저혈압, 고혈압,
실신, 실신, 현기증,
구강, 얼굴, 목, 머리 뒤쪽 통증,
변비, 설사.
11번째 뇌신경의 손상은
머리 회전, 머리 앞쪽 굴곡, 어깨 올림, 견갑골 날개, 어깨 통증의 약화로 이어질 수 있습니다.
12번째 뇌신경의 손상은
말더듬, 혀가 두껍고 무겁거나, 어색한 느낌으로 이어질 수 있습니다.
추가적인 신경 또는 비신경 구조가 관련되어 있다면,
증상은 훨씬 더 광범위할 수 있습니다.
LCN의 통증은
신경통, 감각 이상, 감각 이상 통증, 근육통, 삼킴 통증과 같은 통증과 함께 나타날 수 있습니다.
신경통은
전기 충격과 같은 감각의 발작적인 고통스러운 공격이 특징이며,
특정 유발 부위의 무해한 자극에 의해 자발적으로 발생하거나 유발됩니다.
통증은
일반적으로 하나 또는 여러 개의 신경 분포에서 발생합니다.
LCN의 신경통은
드문 설인두 신경통과 훨씬 더 드문 후두신경 신경통입니다.
설인두 신경통은 귀, 인두, 목, 편도선 또는 혀의 기저부에서 발생하는 극심한 통증 발작이 특징입니다.
통증은 혀가 치아[12] 또는 혀의 뒤쪽과 접촉할 때 유발될 수 있습니다.
설인두 신경통은 반사성 서맥(미주신경 경로를 통한 반사 작용의 원심성 사지)으로 인한 실신(실신)과 관련이 있을 수 있으며, 경우에 따라 삼킴으로 인해 유발될 수 있습니다. 설인두 신경통은 가장 흔하게 일차성(인접한 혈관 고리에 의한 신경 압박)[14] 또는 다발성 경화증[13,15] 또는 독수리 증후군[16]과 같은 내재적 뇌 병변에 이차적으로 발생합니다. 후두 신경통은 드문 질환이며, 편향된 설골[17], 이전 급성 후두염[18], 인두 게실[19], 또는 특발성[20]으로 인해 발생합니다. 환자는 갑상선-설골막 부위에 발작성 찌르는 듯한 통증을 호소하며, 이 통증은 삼킴으로 유발되거나 악화될 수 있습니다[17].
Investigations to Diagnose Lower Cranial Nerve Lesions
Clinical exam (signs of lower cranial nerve lesions)
Neurological
Examination of the 9th cranial nerve includes asking the patient to swallow or cough. In addition, taste and a general sensation of the posterior third of the tongue and the throat can be evaluated. The bitter taste (posterior third of the tongue) is used to test for this nerve. There may also be hypoesthesia of the soft palate or reduced palatal or pharyngeal reflexes. Physical exam of the 10th cranial nerve includes eliciting the gag reflex, to look if the uvula is deviated from the side of the lesion, if there is failure of palate elevation upon phonation, and evaluation of speech impairment. In case of bilateral vagal nerve lesions, heart rate and breath rate may be increased. The somatic sensory function can be assessed by testing the sensibility of the external auditory meatus. The 11th cranial nerve is tested by assessing strength, mass, and spontaneous activity of the trapezius and sternocleidomastoid muscle. The patient is asked to shrug the shoulders with and without resistance. The sternocleidomastoid is tested by asking the patient to turn the head to the left or right or to anteflect the head against resistance. Inspection of the muscles may reveal wasting or fasciculations. The 12th cranial nerve is tested by asking the patient to stick the tongue out straight. If there is a lesion of the nerve, the tongue will deviate toward the affected side or there may be wasting and fasciculations if the lesion is not acute. The strength of the tongue can be tested by pressing it toward the cheek against the finger of the examiner outside. Weakness of the tongue will also manifest as slurred speech. Practical bedside testing is used to identify lesions in the anterior part of the oral cavity (“B”), the oral cavity in particular the tongue (“T”) and the posterior part of the oral cavity (“G”).
Lower cranial nerve lesions may occur in particular distributions giving rise to various syndromes, such as Horner-syndrome, Tapia-syndrome, Collet-Sicard-syndrome, Vernet-syndrome, Jugular-foramen-syndrome, Garcin-syndrome, Schmidt-syndrome, or Villaret's-syndrome [Table 1]. These syndromes are usually case-based descriptions, which are useful, to summarize, the findings, but usually lack accuracy.[21,22,23,24] Vernet-syndrome manifests with dysphonia, hoarseness, drop of soft palate, deviation of the uvula, dysphagia, hypoesthesia of the posterior third of the tongue, reduced parotid gland secretion, loss of gag reflex, and weakness of the sternocleidomastoid and trapezius muscles. Jugular-foramen-syndrome is characterized by acute onset dysphagia, dysphonia, and accompanied or preceded by cranial, cervical, or pharyngeal pain.[25] The most frequent cause of jugular-foramen-syndrome is herpes zoster oticus with herpetic eruptions of the skin or the mucosa. Magnetic resonance imaging (MRI) may reveal contrast enhancement around the jugular foramen, suggesting inflammation of the glossopharyngeal or vagal nerve ganglia.[25] Imaging may also show erosions of bones or metastasis. The diagnosis is confirmed by demonstration of varicella-zoster virus (VZV)-DNA or VZV antibodies in the cerebro-spinal fluid (CSF).[25]
하부 뇌신경 병변 진단을 위한 검사
Lower Cranial Nerve Lesions
임상 검사(하악신경 병변의 징후)
신경학적 검사
9번 뇌신경 검사는
환자에게 삼키거나 기침을 하도록 요청하는 것으로 시작됩니다.
또한, 혀와 목의 뒤쪽 1/3 부분의 미각과 전반적인 감각을 평가할 수 있습니다.
이 신경은 쓴맛(혀 뒤쪽 1/3 부분)을 통해 테스트됩니다.
또한, 입천장의 감각 저하 또는 구개 또는 인두 반사 감소가 있을 수 있습니다.
제10뇌신경의 신체 검사는
구역 반사를 유도하여, 목젖이 병변의 측면에서 벗어난지, 발성 시 구개 상승이 실패하는지, 언어 장애를 평가하는 것을 포함합니다.
양측 미주신경 병변의 경우, 심박수와 호흡률이 증가할 수 있습니다. 체
성 감각 기능은 외이도의 민감도를 테스트하여 평가할 수 있습니다.
11번째 뇌신경은
승모근과 흉쇄유돌근의 힘, 질량, 자발적 활동을 평가하여 테스트합니다.
환자에게 저항 없이 어깨를 으쓱하도록 요청합니다.
흉쇄유돌근은 환자에게 머리를 좌우로 돌리거나 저항을 가해 머리를 앞으로 젖히도록 요청하여 테스트합니다.
근육 검사를 통해 위축이나 근긴장 이상이 발견될 수 있습니다.
12번 뇌신경은
환자에게 혀를 똑바로 내밀어 보도록 하여 검사합니다.
신경에 병변이 있으면 혀가 병변이 있는 쪽으로 치우치거나,
병변이 급성이 아닌 경우 위축과 근긴장 이상이 나타날 수 있습니다.
혀의 힘은 검사자의 손가락으로 혀를 뺨 쪽으로 누르는 방법으로 검사할 수 있습니다.
혀의 약점은 또한 말을 더듬는 형태로 나타납니다.
침대 옆에서 실시하는 실용적인 검사는 구강 앞부분(“B”), 특히 혀(“T”)와 구강 뒤쪽(“G”)의 병변을 확인하는 데 사용됩니다.
특히, 하부 뇌신경의 병변은 다양한 증후군을 유발하는 특정 분포에서 발생할 수 있습니다.
예를 들어,
호너 증후군,
타피아 증후군,
콜레-시카르 증후군,
베르네 증후군,
경정맥개구 증후군,
가르신 증후군,
슈미트 증후군, 또는 빌라레 증후군 등이 있습니다 [표 1].
이러한 증후군은 일반적으로 사례 기반의 설명으로, 결과를 요약하는 데 유용하지만 정확성이 부족합니다. [21,22,23,24] 베르네 증후군은 발성 장애, 쉰 목소리, 뚝뚝 떨어지는 목소리로 나타납니다.
입천장의 연약한 부분,
목젖의 변형,
연하곤란,
혀의 뒤쪽 1/3의 감각 저하,
이하선 분비 감소,
구역반사의 상실,
그리고 흉쇄유돌근과 승모근의 약화.
경정맥동 증후군은
급성 발병성 연하곤란, 발성장애, 두개골, 경추, 인두 통증이 동반되거나 선행되는 것이 특징입니다. [25]
경정맥동 증후군의 가장 흔한 원인은 피부 또는 점막의 대상포진 발진과 함께 발생하는 대상포진성 이염입니다.
자기공명영상(MRI)은
경정맥 주변의 조영 증강을 보여줄 수 있으며,
이는 설인두 신경절 또는 미주신경 신경절의 염증을 시사합니다.25]
영상은 또한 뼈의 침식이나 전이를 보여줄 수 있습니다.
진단은 뇌척수액(CSF)에서 수두 대상포진 바이러스(VZV) DNA 또는 VZV 항체의 검출로 확인됩니다.25]
Table 1.
Syndromes of lower cranial nerve lesions
Oto-rhino-laryngological
Clinical exam by the oto-rhino-laryngologist may be helpful to detect oto-rhino-laryngological causes of LCN lesions. The oto-rhino-laryngologist may assess morphology and function of the oral cavity and the larynx.
Instrumental investigations
Most helpful in the work-up of LCN lesions is the application of static or dynamic imaging methods, such as X-ray (base of the skull, styloid process, deglutition), video-cinematography (dynamic), computed tomography, MRI, angiography (static), or ultrasound [Figure 1]. A useful tool to investigate lesions of the tongue is an ultrasound. Muscle MRI may show atrophy as an indirect sign of a lesion of the supplying nerve by bone erosion or metastases. Oto-rhino-laryngological techniques, such as fiber-optic endoscopy can be helpful to detect causes of LCN lesions. Electrophysiological techniques, such as nerve conduction studies,[26] electromyography (larynx muscles (cricothyroid, thyroarytenoid muscles), sternocleidomastoid, trapezius muscles), electrical nerve root stimulation, or trans-cranial magnetic stimulation (accessory, hypoglossal nerve) are helpful for localizing the level of a nerve lesion and to determine whether it is axonal or demyelinating in nature. Intra-operative electrophysiological monitoring of LCN functions can be helpful to avoid iatrogenic damage of the LCN during surgery. Blood chemistry can be useful to assess inflammatory markers, antibodies against viruses, vasculitis parameters, vitamin levels, tumor-markers, etc. CSF investigations may be helpful to detect infections (meningitis), neoplastic (carcinosis), or immunologic disease (radiculitis) as a cause of LCN lesions. To assess parasympathetic functions autonomic testing by means of the Valsalva maneuver, heart rate variability, tilt test, gastric emptying tests, the sympathetic skin response, the quantitative sudomotor axon reflex test, or the thermoregulatory sweat test is available.
보조적 조사
LCN 병변의 진단에 가장 도움이 되는 것은 X-선(두개골 기저부, 스타일러스 과정, 연하), 비디오-시네마토그래피(동적), 컴퓨터 단층 촬영, MRI, 혈관 조영술(정적), 초음파[그림 1]와 같은 정적 또는 동적 영상 기법의 적용입니다. 혀의 병변을 조사하는 데 유용한 도구는 초음파입니다. 근육 MRI는 뼈 침식이나 전이에 의한 공급 신경의 병변의 간접적 징후로 위축을 보여줄 수 있습니다. 광섬유 내시경과 같은 이비인후과 기법은 LCN 병변의 원인을 감지하는 데 도움이 될 수 있습니다. 신경 전도 연구[26], 근전도 검사(후두 근육(갑상선 갑상선근, 갑상선 아티노이드근), 흉쇄유돌근, 승모근), 전기적 신경근 자극, 또는 경두개 자기 자극(부속, 설측하신경)과 같은 전기 생리학적 기법은 신경 병변의 수준을 국소화하고 그것이 축삭성인지 탈수초성인지 판단하는 데 도움이 됩니다. 수술 중 LCN 기능의 전기 생리학적 모니터링은 수술 중 LCN의 의인성 손상을 방지하는 데 도움이 될 수 있습니다.
혈액 화학 검사는 염증 지표, 바이러스에 대한 항체, 혈관염 지표, 비타민 수치, 종양 표지자 등을 평가하는 데 유용할 수 있습니다. 뇌척수액 검사는 LCN 병변의 원인인 감염(수막염), 종양(암종), 또는 면역 질환(신경근염)을 감지하는 데 도움이 될 수 있습니다.
부교감 신경 기능을 평가하기 위해
발살바 기동,
심박수 변동성,
경사 테스트,
위 배출 테스트,
교감 피부 반응,
정량적 발한성 축삭 반사 테스트,
체온 조절 땀 테스트를 이용한 자율 신경 테스트가 가능합니다.
Figure 1.
Work-up in case of symptoms indicating lower cranial nerves lesion
Disorders of Lower Cranial Nerves
Isolated affection of a single cranial nerve is rare [Table 2]. In the majority of the cases, multiple LCN are simultaneously affected. Causes of LCN lesions can be genetic, vascular, traumatic, iatrogenic, infections, immunologic, metabolic, nutritional, degenerative, or neoplastic.
하부 뇌신경 장애
단일 뇌신경의 단독 장애는 드뭅니다 [표 2].
대부분의 경우, 여러 개의 LCN이 동시에 영향을 받습니다.
LCN 손상의 원인은 유전적, 혈관적, 외상적, 의인성, 감염성, 면역성, 대사성, 영양성, 퇴행성 또는 종양성일 수 있습니다.
Table 2.
Lower cranial nerve lesions
Genetic causes
The most frequent genetic disorders associated with LCN involvement are the motor neuron disorders (MNDs). They include amyotrophic lateral sclerosis (ALS), spinal muscular atrophy, bulbospinal muscular atrophy (BSMA), and unclassified MNDs (Sandhoff disease, triple-A-syndrome, Brown-Vialetto-Van Lare-syndrome). Among these, LCN are affected in ALS, BSMA, triple-A-syndrome, and Brown-Vialetto-Van Lare-syndrome. More rarely, cranial nerves are involved in hereditary neuropathy.
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis is the most common among the MNDs.[27,28,29] ALS is sporadic in the majority of the cases but hereditary in an increasing number of patients with sporadic ALS.[29] More details are thus presented in the chapter about degenerative causes.
Bulbospinal muscular atrophy
Bulbospinal muscular atrophy (Kennedy disease) is a rare X-linked MND with onset in adolescence or early adulthood. BSMA is caused by a CAG-triplet repeat expansion in the androgen-receptor gene.[30] Clinically, BSMA is characterized by slowly progressive weakness and wasting of all muscles with a predominance of the limb muscles, impairment of speech and swallowing, and muscle cramps. In addition to motor neuropathy, there is also sensory neuropathy, and postural and intention tremor. Nonneurological manifestations include gynecomastia, loss of masculinisation, and erectile dysfunction. Motor manifestations may be asymmetric. Lifespan is usually normal. Leuprorelin and symptomatic therapy is available.[30]
Brown-Vialetto-Van Lare-syndrome
Brown-Vialetto-Van Lare-syndrome is a rare, hereditary disorder characterized by bulbar palsy, respiratory compromise, and sensorineural hearing loss.[31] Brown-Vialetto-Van Lare-syndrome is due to mutations in the SLC52A1 gene encoding the human riboflavin transporter-1.[31] Substitution of riboflavin has a beneficial effect and prolongs life expectancy.[31]
Triple-A-syndrome
Triple-A-syndrome is a rare condition mimicking MND. It follows an autosomal recessive trait of inheritance and is due to mutations in the ALADIN gene, which encodes a nuclear pore complex component.[32] Clinically, triple-A-syndrome is characterized by esophageal achalasia, alacrimia, adrenal insufficiency, and progressive bulbar spinal muscular atrophy with the involvement of upper and lower motor neurons.[32]
Hereditary neuropathies
Rarely, mononeuropathy of the accessory nerve may be a manifestation of hereditary neuropathy with liability to pressure palsies (HNPP).[33] Another LCN involved in HNPP is the hypoglossal nerve.[34] The affection of the hypoglossal nerve has been also reported in other types of hereditary neuropathy.[35] Dysarthria may be a feature of hereditary motor and sensory neuropathy Lom.[36] In X-linked Charcot-Marie-Tooth disease due to mutations in the GJB1 gene, neuropathy may be associated with vocal cord paralysis.[37] Vocal cord paralysis has been also reported in congenital hypomyelination.[38]
Vascular
Stroke
Ischemic stroke is a frequent cause of LCN affection, particularly if nuclei or fascicles of any of the four LCN are affected (nuclear or fascicular cranial nerve lesions). Additionally, LCN may be secondarily affected in case of supranuclear vascular lesions [Table 3]. According to the location of the ischemic lesion in the brain stem, a dorsal, lateral, paramedian, or median location of the vascular lesion is delineated. In addition, short, long, or circumferential arteries may be involved. Various syndromes are differentiated of which the Wallenberg-syndrome (lateral medullary syndrome) is the most common [Table 1]. More rare ischemic medullary stroke syndromes include the Avellis-syndrome, the Jackson-syndrome, and the Opalski-syndrome [Table 1].
Table 3.
Differentials of LCN lesions (disorders which indirectly affect LCN or are associated with LCN lesions)
Aneurysm
Internal carotid artery aneurysm directly underneath the base of the skull may affect the 9th, 10th, 11th, or 12th cranial nerves.[39] These nerve lesions may result in impaired swallowing or dysarthria but also tongue weakness.[39] Treatment of such aneurysms by parent vessel occlusion may result in shrinkage of the aneurysm and consecutive improvement of the neurological deficits.[39] Additionally, aneurysms of the intracranial vertebral artery may be associated with LCN neuropathy.[40] Aneurysms of the posterior inferior cerebellar artery (PICA) may affect the glossopharyngeal, the vagal, or the accessory nerve.[41]
Artery dissection
Quite frequently internal carotid artery dissection can lead to compression of the hypoglossal nerve.[11,42] Also, dissection of the vertebral artery may go along with hypoglossal palsy.[43] Dissection of the internal carotid artery may even lead to Villaret's-syndrome[44] or Collet-Sicard-syndrome. Vertebral artery dissection may cause Tapia-syndrome [Table 1].[45] Dissection of the internal carotid artery may result in dysphonia and homolateral shoulder paralysis due to palsy of the vagal and accessory nerves.[46] Horner-syndrome may be another manifestation of a carotid artery dissection, which usually is painful.
Vasculitis
Rarely, Takayasu vasculitis may lead to injury of the LCN, particularly the glossopharyngeal or the hypoglossal nerves.[47] There is one report about a patient who developed glossopharyngeal neuralgia following an arteritis temporalis.[48] Another patient developed Vernet-syndrome [Table 1] from giant cell arteritis.[49] Collet-Sicard-syndrome [Table 1] has been reported in a single patient with panarteriitis nodosa.[50]
Ischemia of lower cranial nerves
Since LCN receive their arterial supply from a network of tributaries originating from the internal carotid, external carotid and the vertebro-basilar arteries, it is conceivable that impaired blood supply via these networks results in ischemia of the supplied nerves.[51] Small vessel disease, as well as macroangiopathy, may affect the vasa nervorum of LCN and may thus lead to ischemia causing LCN dysfunction.[51] The vascular supply of LCN is also of relevance for the base of the skull surgery[51] and an issue in interventions by artery embolization in tumor therapy.
Rare vascular causes
A rare vascular cause of LCN compromise is brainstem cavernoma.[52] Lesion of the LCN may persist despite surgery.[53] A further rarity of a vascular cause is transient dysarthria due to compression of the hypoglossal nerve by a calcified persistent hypoglossal artery.[54]
TraumaBase of the skull fracture
The base of the skull fractures frequently affect the LCN, particularly if the occipital condyles are involved.[55] The outcome of the base of the skull fractures is poor particularly if LCN are affected.[56] Isolated palsies of the LCN may occur in case of occipital condyle fractures.[57,58] Ipsilateral[59] or contralateral[60] hypoglossal nerve palsy is the most frequent isolated LCN injury from condyle fractures. Early full recovery of these lesions has been reported,[57] but in other cases, palsy persisted for months. Traumatic LCN lesions may not only manifest immediately after the trauma, but with a delay of several days.[61] Prognosis of traumatic LCN lesions is poor if the carotid artery is additionally damaged.[62]
Atlas fractures
Atlas burst fractures rarely cause neurological deficits because of an enlarged opening of the spinal canal at this level and a tendency of the lateral masses to move centrifugally away from the cord posttraumatically.[63] In case of atlas fracture and congenital basilar invagination, however, LCN may be also damaged.[63] Though cranial nerve lesions from atlas fractures are rare, they occasionally occur and affect the 9th through 12th cranial nerve.[64] The most frequent clinical manifestation of cranial nerve involvement is dysphagia, which resolves upon Halo-fixation.[65]
Traumatic brain injury
Traumatic brain injury (TBI) is a leading cause of death or disability, particularly in the younger population.[66] LCN lesions may be a complication of TBI.[67] Decompression of injured cranial nerves improves the outcome of TBIs.[67] Injury of the vagal nerve may increase vagal tone, which consecutively may diminish the anti-inflammatory capacity of patients with TBI. In the acute phase of TBI patients are thus more vulnerable to infectious disease due to decreased immune response.[66]
Fracture of the styloid process
Fracture of the styloid process may lead to compromise of the 9th cranial nerve.[68] The glossopharyngeal nerve is compromised because it crosses the gap between the process and the first cervical vertebrum.[68] Clinical manifestations of a styloid fracture include neck pain or painful dysphagia.[68] Not only bone fractures, but also traumatic soft tissue lesions may cause LCN dysfunction.
IatrogenicCarotid surgery
Carotid surgery may go along with the iatrogenic injury of a single or multiple LCN.[69] Local pressure may lead to impairment of various functions of the 9th or 12th cranial nerves. Particularly during the open restoration of the internal carotid artery, LCN may be iatrogenically injured.[69] In a retrospective study of 543 patients undergoing carotid surgery the glossopharyngeal nerve was damaged in 2 cases (0.4%), the vagal nerve in 9 cases (1.8%), and the hypoglossal nerve in 7 cases (1.4%).[69] Most of these lesions recovered spontaneously and completely.[69] In a retrospective study of 752 patients undergoing reconstructive surgery of the supra-aortic arteries damage of the glossopharyngeal nerve occurred in 16 cases with complete recovery during a period of 1–6 months.[70]
Neck dissection
Neck dissection refers to a surgical procedure in which the fibro-fatty contents of the neck including cervical lymph nodes are removed for the treatment of cervical lymphatic metastases. It is also applied to malignancies of the skin (head, neck), the thyroid, or the salivary glands. Complications of the procedure affecting LCN include damage of the hypoglossal or accessory nerve. To avoid damage of the accessory nerve from neck dissection, monitoring of accessory function during surgery is increasingly performed.[71]
Rare iatrogenic causes
Rare iatrogenic causes of LCN lesions include unilateral taste disturbance due to local pressure of a classical laryngeal mask onto the nerve,[72] glossopharyngeal or hypoglossal nerve palsy after tonsillectomy, or vagal nerve palsy after obesity treatment.[73] During bariatric surgery the vagal may be accidentally compromised without severe consequences.[74] Thyroid surgery may be complicated by injury of the accessory and recurrent nerve.[75] Rhinoplasty may be complicated by Tapia-syndrome.[76]
InfectiousVaricella-zoster virus
Varicella-zoster virus infection involving the LCN may manifest as Ramsey-Hunt-syndrome, Garcin-syndrome, or Jugular-foramen-syndrome. Ramsey-Hunt-syndrome is caused by infection with the virus of the geniculate ganglion of the facial nerve.[77] Clinically, it is characterized by 7th cranial nerve palsy, otic pain, and herpetic vesicles around the auricle and external auditory canal.[77,78] Occasionally, LCN may be additionally involved, particularly the vagal nerve which may manifest with transient dysphagia.[77,78] Jugular-foramen-syndrome is characterized by acute onset dysphagia, dysphonia, and accompanied or preceded by cranial, cervical, or pharyngeal pain.[13] The most frequent cause of Jugular-foramen-syndrome is herpes zoster oticus with herpetic eruptions of the skin or the mucosa. MRI may reveal contrast enhancement around the jugular foramen, suggesting inflammation of the glossopharyngeal or vagal nerve ganglia.[13] The diagnosis is confirmed by demonstration of VZV-DNA or VZV-antibodies in the CSF [Figure 1].[13] If there is unilateral herpes zoster infection of the larynx, ipsilateral LCN may be affected.[79] In rare cases VZV-infection may present as Garcin-syndrome.[80]
Diphtheria
Diphtheria is characterized by initial infection of the upper airway tract with Corynebacterium diphteriae, which later is secondarily complicated by systemic radiculoneuropathy also affecting the 9th or 10th cranial nerve. Clinically, these patients present with paralyzed accommodation, palatal paralysis, tongue weakness, and sensory > motor polyneuropathy diphtheria is frequently associated with cardiomyopathy and arrhythmias. Paralysis of the diaphragm occurs in 1/3 of the cases. In severe cases laryngospasm may develop. Autonomic involvement includes sinustachycardia, bladder dysfunction, or arterial hypotension. Occasionally, there may be facial weakness, ophthalmoparesis, or optic atrophy.
Poliomyelitis
Poliomyelitis is caused by an RNA-enterovirus and occurs in only 5–10% of the infected patients. It starts with fever, myalgia, and nonspecific gastrointestinal symptoms. 4–20 days after the infection patients develop meningitis with headache and stiff neck followed by weakness of limb muscles.[81] In 20% of the cases, poliomyelitis is associated with bulbar involvement.[81] Cranial nerves are variably affected. If the 7th cranial nerve is affected, hemifacial atrophy may ensue.[82] In other cases, the 5th, 9th, 10th, and 11th cranial nerves may be involved.[83] Skeletal muscles supplied by these nerves become atrophic.[83]
Tetanus
Tetanus is an infection caused by the endotoxins of Clostridium tetani, which block the presynaptic SNARE protein. It may occur focally or generalized. Entry of the toxin into the body is in 75% of the cases an acute wound. Despite modern medicine, mortality still ranges between 15% and 30%. Among the cranial nerves, the facial nerve is most commonly affected. Others are the nerves supplying the extra-ocular muscles, or the hypoglossal nerve. Rarely, the 9th or 10th cranial nerve is affected.[81]
Botulinism
Concerning the entrance of the bacterium into the body, foodborne botulism and wound botulism are differentiated. Concerning age at onset, infant botulism (in one fifth of the cases via ingestion of honey) and adult intestinal toxaemia are differentiated. Accidentally, botulism may be caused by overdosing therapeutic botulinum toxin. In rare cases Clostridium botulinum infection may involve cranial nerves.[84] Particularly hemorrhaghe within the vagal nerve has been reported.[84]
Borreliosis
Borreliosis and Lyme disease are due to an infection with the spirochete Borrelia burgdorferi. In the majority of the cases it is transmitted from ticks to humans. Occasionally, the central nervous system (CNS) is affected manifesting as headache, sleep disturbance, papilledema, meningitis, meningo-radiculitis, myelitis, affection of the cranial nerves, ataxia, chorea, and altered mental state. LCN are rarely involved.[85] Manifestations attributable to LCN involvement resolved upon adequate antibiotic treatment in a single patient.[85]
Tuberculous meningitis
Affection of the LCN by tuberculous meningitis is rare and usually has a poor outcome.[86] Involvement of the LCN in tuberculous meningitis may manifest clinically as speech disturbance (slurring).[86] If tuberculous spondylitis involves the craniocervical junction, affected patients may present with isolated hypoglossal nerve palsy.[87]
Immunological
Sarcoidosis
Sarcoidosis is an inflammatory, granulomatous, systemic disease. In about 25% of the cases involvement of the nervous system occurs (neurosarcoidosis).[88] Involvement of the cranial nerves is the most common abnormality in neurosarcoidosis and most frequently affects the 7th and second most frequently the 2nd cranial nerve. Only rarely, the 8th, 9th, or 10th cranial nerves are being affected. In a retrospective study of 54 patients with neurosarcoidosis, those with LCN involvement had a slightly better outcome than those with optic nerve involvement.[88] Clinical manifestations of neurosarcoidosis usually resolve upon administration of steroids.[89]
Multiple cranial nerve neuropathy
Multiple cranial nerve neuropathy is the pharyngo-facial variant of Guillain-Barre-syndrome (GBS).[90] Affected patients present with facial swelling, bilateral facial palsy, and bulbar palsy with dysphagia or aspiration.[90] There may also be weakness of the neck, the upper limbs, the hips, or the facial muscles. In 70% of the cases, an upper respiratory tract infection and in 30% diarrhea or other gastrointestinal problems precede the immune neuropathy. Infectious agents are the campylobacter jejunii or the cytomegaly virus. Intravenous immunoglobulins have a beneficial effect. In a case of Miller-Fisher-syndrome associated with Bickerstaff encephalitis, the patient presented with ophthalmoplegia, bilateral facial palsy, dysphagia, dysarthria, neck weakness, distal quadriparesis, and ataxia.[91] These abnormalities improved gradually upon intravenous immunoglobulins.[91]
Pharyngo-cervical-brachial variant of Guillain-Barre-syndrome
This GBS variant manifests with dysphagia, weakness of facial muscles, neck flexors, and proximal upper limb muscles, ophthalmoplegia, ataxia, and autonomic dysfunction (heart rate, bladder). Laboratory and electrophysiological investigations are similar to those in GBS.
Chronic inflammatory demyelinating polyneuropathy
Rarely, LCN are involved in chronic inflammatory demyelinating polyneuropathy (CIDP) manifesting as hypogeusia due to the involvement of the glossopharyngeal nerve.[92] Much more frequently than the glossopharyngeal nerve, the hypoglossal nerve may be affected in CIDP patients.[93]
Multiple sclerosis
Neuropathic pain, manifesting as cranial neuralgia, is a frequent feature of multiple sclerosis.[94] The most well-known LCN neuralgia is the one of the glossopharyngeal nerve. Demyelination in the centrally myelinated part of the cranial nerve roots is held responsible for the development of pain.[94] The most frequent neuralgia of the LCN in multiple sclerosis is glossopharyngeal neuralgia.[15]
Metabolic
Diabetes
Autonomic neuropathy from diabetes may also affect the vagal nerve and its branches and may be associated with cardiovascular and gastrointestinal compromise.[95] In humans or animals with severe hyperglycemia, impairment of the cardiac autonomic nervous system may occur.[96,97] Diabetic gastroparesis is well-known but poorly understood phenomenon, which may also be due to affection of the autonomic innervation by the vagal nerve.[98]
Nutritional
Vitamin B12 deficiency
Rarely, Vitamin-B12 deficiency may be the cause of unilateral vocal cord palsy.[99] Among cranial nerves, the optic nerve is the one most frequently affected in B12-deficiency.[99] Folate deficiency has not been reported as a cause of LCN lesions. Autonomic dysfunction with vagal withdrawal and defective sympathetic activation has been reported in patients with cobalamin deficiency.[100]
Degenerative
Amyotrophic lateral sclerosis
Usually, ALS manifests clinically in adulthood as a continuum between the exclusive affection of the upper motor neurons and exclusive affection of lower motor neurons.[29] The affection of the LCN leads to a bulbar syndrome including slurred and dysarthria and dysphagia. LCN may be affected already at the onset of the disease (bulbar-onset ALS), or following the affection of the limb muscles (limb-onset ALS). In ALS with bulbar involvement, a dynamic collapse of the airway due to the gravitational pull of the mandible (mandibula ptosis) may occur in the supine position.
Facial onset sensory and motor neuronopathy
Facial onset sensory and motor neuronopathy is a rare, slowly progressive neurodegenerative disorder clinically characterized by numbness and paresthesias initially in a trigeminal nerve distribution followed by spreading to the scalp, neck, upper trunk, and upper limbs.[101] Later in the course, patients develop muscle cramps, fasciculations, muscle weakness and wasting, dysphagia, and dysarthria.[101] Nerve conduction studies reveal a caudally descending sensorimotor neuropathy.[101] An autopsy may show loss of motoneurons in the hypoglossal nucleus and cervical anterior horns.
Sandifer's syndrome
Sandifer's syndrome is a gastrointestinal disorder characterized by reflex torticollis following deglutition in patients with gastro-esophageal reflux or hiatal hernia.[102] Sandifer's syndrome is regarded as a vagal reflex triggered by afferent impulses transmitted via general visceral afferents to the nucleus tractus solitarii and from there to the nucleus ambiguus and the nucleus dorsalis nervi vagi. From the latter, visceral efferents reach the accessory nerve via hypothetical anastomoses to innervate the trapezius and sternocleidomastoid muscles.[102]
Eagle-syndrome
Eagle-syndrome, also known as syndrome of the elongated styloid process, is characterized by cervical pain, which can be enhanced by head rotation, chewing, or head extension.[103] Neck or throat pain usually radiates to the ipsilateral ear. Other symptoms include foreign body sensation, dysphagia, or facial pain.[104] Symptoms are due to an elongated styloid process or an ossified stylohyoid ligament. The ossification of the ligament leads to throat symptoms due to the affection of the glossopharyngeal nerve.[103]
Chiari malformation
In rare cases, Chiari-I malformation may be associated with the affection of the hypoglossal nerve.[105] More frequently, Chiari-I malformation causes glossopharyngeal neuralgia.[106] Chiari-I malformation may also compromise the vagal nerve. Chiari-I malformation may lead to compression of the LCN between the PICA and the herniated cerebellar tonsil.[107]
Neoplasms
A number of benign or malignant intra- or extra-cranial neoplasms are associated with lesions of the LCN (base of the skull syndromes). Also, bone metastases of the skull base may damage LCN.[108] Intra-cranial neoplasms may be further divided into those located intra-cerebrally and those located extra-cerebrally. Among the extracranial neoplasms, those located just below the skull and those of the neck are differentiated. Intrinsic brain tumors may affect the nuclei or intra-parenchymatous nerve tracts. Malignant spread within the CSF space affects cranial nerves and nerve roots. Tumors affecting the cranial nerves outside the bony skull may spread in an antero-or retrograde fashion.
Intra-cerebral neoplasms affecting nuclei of LCN include the posterior fossa ependymoma,[109] choroid plexus papilloma,[110] primary CNS lymphoma,[111] or rarely metastasis.[112] Extra-axial neoplasms affecting the LCN include cisternal meningeomas,[113] extra-axial cavernomas,[114] choroid plexus papillomas,[115] intracranial Schwannomas,[116] or metastasis.[117] Leptomeningeal carcinomatosis involving the LCN is less frequent than involvement of upper cranial nerves.[118] Suspected leptomeningeal carcinomatosis may not always be confirmed at autopsy. Usually, however, an autopsy in meningeal carcinomatosis may show squamous-type carcinoma cells scattered in the cerebro-spinal leptomeninges and perineurium of almost all spinal and cranial nerve roots, causing severe axonal degeneration.[119] Involvement of the leptomeninges in lymphomatoid granulomatosis may also damage LCN.[120] Bone metastases of the base of the skull, which are usually painful, are a frequent cause of LCN dysfunction including Collet-Sicard-syndrome.[108,121]
The most frequent neoplasms of the jugular foramen are the paragangliomas,[113] followed by Schwannomas,[122] neuroendocrine carcinoma,[123] and meningeomas.[124] Paraganglioma accounts for <1% of neoplasms in the head-neck region. Traditionally, paragangliomas are considered as benign, slowly growing, locally invasive, encapsulated and highly vascularised tumors.[22] Paragangliomas of the neck may even cause Collet-Sicard-syndrome.[22] Neoplasms originating from one of the LCN include the hypoglossal Schwannoma,[125] the vagal nerve Schwannoma,[126] and the neurilemmoma of the vagal nerve.[127] The base of the skull neoplasms affecting LCN include the hemangiopericytoma,[128] the glomus tumor,[129] chordomas,[130] osteosarcoma, or metastasis. Neoplasms of the neck may also injure LCN. Recurrent squamous carcinoma metastatic to the neck may directly infiltrate the glossopharyngeal or vagal nerves.[131] Neck fibrosarcoma as well as multiple myeloma, hypoglossal Schwannoma, jugular foramen neurinoma, jugular foramen metastatic melanoma, or hemangiopericytoma may cause Collet-Sicard-syndrome. Tonsillar carcinoma invading the parapharyngeal space may lead to hypersensitivity of the carotid sinus coupled with glossopharyngeal neuralgia.[132] LCN lesions may also originate from perineural spread of a facial squamous cell carcinoma.[133] In a report of 3 cases, super-selective arterial embolization of paragangliomas with ethylene vinyl alcohol resulted in damage of the 10th, 11th, or 12th cranial nerve with recovery in only one patient.[134]
Conclusions
This review about disorders of the LCN shows that causes of LCN lesions are heterogeneous. Causes of LCN lesions may be classified as genetic, vascular, traumatic, iatrogenic, infectious, immunologic, nutritional, metabolic, degenerative, or neoplastic. LCN may be affected as a single nerve or as multiple LCN. LCN may be affected together with more proximal cranial nerves, together with CNS disease, or together with nonneurological disorders. There are disorders which specifically affect LCN and multisystem disorders with nondominant LCN involvement [Table 4]. If LCN lesions are suspected upon typical symptoms and signs, specific instrumental investigations may confirm the suspected LCN lesion [Figure 1]. Affection of multiple LCN is much more frequent than the affection of a single LCN. Treatment of LCN lesions depends on the underlying cause. An effective treatment is available in the majority of the cases but the requirement for complete recovery is the prompt and correct diagnosis. To correctly assess complaints resulting from LCN lesions, precise knowledge about the anatomy and physiology of the area is a prerequisite.
결론
이 LCN 장애에 대한 검토는 LCN 병변의 원인이 이질적이라는 것을 보여줍니다. LCN 병변의 원인은 유전적, 혈관적, 외상적, 의인성, 감염성, 면역학적, 영양학적, 대사적, 퇴행성 또는 종양성 원인으로 분류될 수 있습니다.
LCN은
단일 신경 또는 다중 LCN으로 영향을 받을 수 있습니다.
LCN은
더 근위부 두개골 신경과 함께, 중추신경계 질환과 함께, 또는 비신경학적 장애와 함께 영향을 받을 수 있습니다.
LCN에 특이적으로 영향을 미치는 장애와 LCN이 비우세하게 관여하는 다중 시스템 장애가 있습니다 [표 4].
전형적인 증상 및 징후로 LCN 병변이 의심되는 경우, 특정 도구 검사를 통해 의심되는 LCN 병변을 확인할 수 있습니다 [그림 1].
다발성 LCN의 병변은 단일 LCN의 병변보다 훨씬 더 빈번하게 발생합니다.
LCN 병변의 치료는 근본적인 원인에 따라 달라집니다.
대부분의 경우 효과적인 치료가 가능하지만, 완전한 회복을 위해서는 신속하고 정확한 진단이 필요합니다.
LCN 병변으로 인한 증상을 정확하게 평가하기 위해서는 해당 부위의 해부학과 생리학에 대한 정확한 지식이 필수적입니다.
Table 4.
Disorders specifically affecting a LCN and disorders with LCN involvement but diagnosed upon systemic manifestations
Footnotes
Source of Support: Nil.
Conflict of Interest: None declared.
References
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