Isolated cranial nerve palsies in multiple sclerosis

Data on patients with multiple sclerosis and cranial nerve involvement as a presenting sign or a sign of disease exacerbation were retrospectively analyzed. Isolated cranial nerve involvement was present in 10.4% out of 483 patients, either as a presenting symptom (7.3%) or a symptom of disease relapse (3.1%). Trigeminal nerve was most frequently involved, followed by facial, abducens, oculomotor and cochlear nerves. Only 54% of patients had brainstem MRI lesion that could explain the symptoms. As multiple sclerosis is a disease characterized by multiple neurological symptoms, while early diagnosis and therapy are critical for the prognosis and course of the disease, the diagnosis of multiple sclerosis should be considered in young adults with cranial nerve involvement.     

Non-Hodgkin's malignant lymphoma of the peripheral nervous system: clinicopathological correlations in ten patients

INTRODUCTION: Identifying tumor infiltration or compression in patients with non-Hodgkin's malignant lymphoma presenting peripheral neuropathy can be a difficult task. METHODS: We collected a series of patients with peripheral neuropathy with demonstrated lymphomatous infiltration or compression managed between October 1977 and October 2001 to search for clinico-pathological correlations. RESULTS: Ten cases were reviewed. Neurological manifestations were the inaugural symptom of the disease in 7 patients. Clinical presentations included 5 focal (3 cranial nerve palsies, 2 brachial radiculopathies) and 5 diffuse neuropathies (3 polyradiculoneuropathies, 1 polyneuropathy and 1 mononeuritis multiplex). The mechanisms of peripheral nerve involvement were classified into lymphomatous meningoradiculitis (5 cases), involvement of cranial nerves or spinal roots in their extraneuraxial course (3 cases) and infiltration of distal peripheral nerves (2 cases). Four long lasting survivals after treatment were observed. CONCLUSIONS: Prognosis depends much more on the haematological disease than on the neurological symptoms or tumor location.     

Cranial nerve palsies accompanying pituitary tumour

We reviewed 12 patients with pituitary tumour and cranial nerve palsy to analyse the clinical characteristics, the radiographic appearances, and the outcome after surgery. All patients had pathologically nonfunctioning macroadenomas with evidence of apoplexy. The third cranial nerve was the most frequently affected, followed by the sixth and fourth cranial nerves. Third cranial nerve palsy manifested as a symptom sequence comprising mydriasis, followed by limitation of gaze and ptosis. These symptoms recovered in reverse order of development. The time taken for recovery of cranial nerve palsy after surgery was significantly correlated with the length of time between the onset of symptoms and surgery. Pituitary apoplexy appears to be the primary cause of cranial nerve palsy with pituitary tumour. Early surgical intervention is most likely to bring about rapid recovery from cranial nerve dysfunction.     

Ophthalmoplegic migraine: inflammatory neuropathy with secondary migraine?

BACKGROUND: This critical review provides a summary of the clinical presentation, neuroimaging, treatment and prognosis in pediatric ophthalmoplegic migraine (OM). The features of OM are not in keeping with its classification as a migraine-variant. METHOD: We review 3 new and 37 reported pediatric OM cases. RESULTS: Headache was an inconsistent feature, with 25% patients showing no evidence of pain at the initial OM episode. Patients demonstrated: 1) prolonged time for symptom resolution to occur (median time 3 weeks); 2) tendency for recurrent episodes to have more severe and persistent nerve involvement; 3) evidence of permanent neurological sequelae with recurrent episodes (30% of patients); 4) rapid improvement and shortened duration with corticosteroid therapy and; 5) transient, reversible MRI contrast enhancement of the affected cranial nerve (86% of patients). These features would not be expected in primary migraine headache. CONCLUSION: A detailed understanding of the natural history of OM is essential for the clinical. This review provides support that OM may result from cranial nerve inflammation with headache a secondary and later feature of this condition.     

Cranial nerve involvement in patients with leprous neuropathy

BACKGROUND: Leprosy is one of the most common causes of peripheral neuropathy, perhaps closely matched by diabetic neuropathy. Patterns of peripheral neuropathy in leprosy can be varied, which may include mononeuropathy, mononeuritis multiplex and symmetric polyneuropathy. Cranial nerves, especially facial and trigeminal nerves, are also commonly involved in leprosy. AIMS: To find out the pattern and spectrum of cranial nerve involvement in a consecutive series of patients with leprous neuropathy. SETTINGS AND DESIGN: A retrospective review of patients admitted with leprosy to the Neurology Department of a tertiary care center. MATERIALS AND METHODS: All consecutive patients admitted during an 8-year period (1995-2003) and diagnosed to have leprosy were included. They were clinically evaluated to determine the frequency and pattern of cranial nerve involvement. RESULTS: About 18% (9/51) of the leprosy patients seen during that period had clinical evidence of cranial nerve involvement. Facial and trigeminal nerves were the most commonly affected (five and four patients respectively). CONCLUSIONS: Cranial nerve involvement is common in leprosy, which emphasizes the need to carefully examine them. Also, one should exclude leprosy in patients presenting with isolated cranial neuropathies.     

Cranial nerve involvement in childhood polyarteritis nodosa.

Amongst a variety of neurological manifestations of childhood polyarteritis nodosa, cranial nerve involvement is unusual. We report 4 cases with cranial nerve palsies in a series of 36 biopsy-proven patients. Two cases presented with IIIrd nerve palsy alone, one with right IIIrd and left IVth nerve palsy, and one with peripheral VIIth nerve paresis. All 4 patients showed good response to prednisolone and cyclophosphamide treatment. Cranial nerve involvement in childhood polyarteritis nodosa seems not so rare when patients are followed on long term basis.     

重症吉兰-巴雷综合征的预测因素分析

目的分析轻症与重症吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)患者临床及神经电生理检查特点,筛选出重症患者的预测因素。方法回顾性分析2006年1月至2015年11月在本院住院治疗的GBS患者101例。根据疾病高峰期Hughes评分划分为轻症组(0~2分)和重症组(3~6分)。分别统计两组患者发病年龄、性别、前驱感染史、首发症状、是否有延髓功能障碍、是否累及颅神经和自主神经、有无轴索损害等指标,分析两组之间差异,并筛选出重症患者的预测因素。结果以非感觉异常为首发症状(P0.001)、有延髓功能障碍(P0.001)、颅神经受累(P=0.025)、自主神经系统受累(P=0.018)、运动系统受累(P=0.004)以及轴索损害(P0.001)的患者容易发展为重型患者。多因素Logistic回归分析发现轴索损害(P=0.008,OR=4.632)、延髓受累(P=0.010,OR=10.420)、颅神经受累(P=0.047,OR=0.076)是发展为重型的独立危险因素。结论轴索损害、延髓受累和颅神经受累可能是GBS患者发展至重型的有意义的预测因素。     

BRAINSTEM AND CRANIAL NERVE INVOLVEMENT IN MULTIPLE SCLEROSIS

Nation-wide surveys in Israel in 1960 and 1966 disclosed 295 patients with multiple sclerosis (MS). Brainstem signs were found in 14 per cent at the onset of the disease and 65 per cent during the course of illness. Cranial nerves supplying extraocular muscles were the most commonly affected. Development of cranial nerve signs was not influenced by sex, age at onset, duration of illness or ethnic origin. Patients with cranial nerve signs tended to have a remittent course. Patients with cranial nerve involvement had a mortality similar to those without such involvement. However, bulbar involvement was associated with an increased death rate. The literature on trigeminal and glossopharyngeal neuralgia, facial paralysis and deafness as well as other cranial nerve involvement in MS is reviewed.     

Perineural spread of cutaneous head and neck cancer. Its orbital and central neurologic complications

The ability of cutaneous squamous cell carcinoma of the face to cause ophthalmoplegia or central nervous system dysfunction via perineural spread is not well recognized. Five patients presenting to a general neurology unit are described in whom partial or complete ophthalmoplegia developed following fifth and seventh cranial nerve involvement by cutaneous squamous cell carcinoma. Two patients subsequently developed a contralateral hemiparesis; and one, multiple cranial nerve palsies as the tumor spread centrally. Normal radiologic findings or complete healing of the primary skin lesion caused delay in the diagnosis in three of the patients. When ophthalmoplegia or central nervous system dysfunction develops as a consequence of perineural spread of cutaneous facial cancer, management is palliative.     

MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature

Background: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. Case series: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain–Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness. Cranial nerve enhancement was seen in 6/17 patients and optic nerve/chiasm enhancement was seen in 3 patients. Conclusion: Cranial nerve enhancement and optic pathway in particular, can be seen in patients with MFS. Imaging findings do not always correlate with clinical manifestations of cranial nerve involvement.     

Paralysis of the 3d cranial nerves in diabetes and common oculomotor vascularization]

The purpose of this paper is to present explanations for various patterns of IIIrd cranial nerve involvement in diabetes mellitsus, based on its vascularisation. Three clinical cases of diabetes with numerous attacks of IIIrd nerve paralysis are reported. The following patterns were observed: (1) isolated; (2) associated with Vth nerve involvement and; (3) associated with invovlement of the Vth, IVth, VIth, and occasionally IInd and VIIth nerves. An anatomical study of IIIrd nerve vascularisation demonstrates three territories which could correspond to the 3 patterns of clinical expression. The arterial branches to the IIIrd nerve give off no collaterals in the posterior region of the circle of Willis. In the supra-cavernous region, vascularisation of the IIIrd nerve may be associated with that of the IVth. Eventually, vascularisation of the IIIrd nerve in the intracavernous region is associated with that of the Vth, IVth, VIth, and occasionally IInd and VIIth cranial nerves. Thus, a painless paralysis of the IIIrd nerve (isolated or associated only with an involvement of the IVth) would predictably be related to a vascular disturbance limited to the first or second portion of this nerve, whereas a painful paralysis of the IIIrd nerve, without or with associated involvement of other cranial nerves, would relate to a vascular distrubance in the intra-cavernous region. The anatomo-clinical relationships that have been presented: (1) support the vascular basis of IIIrd nerve paralysis in diabetes; (2) explain the various clinical patterns of IIIrd nerve involvement in that disorder and; (3) act as a model which can be applied to the study of ischemic pathology in other cranial nerves and other etiologies.     

The incidence and course of paraneoplastic neuropathy in women with epithelial ovarian cancer

Summary Sensorimotor polyneuropathy is the most common of the paraneoplastic syndromes involving the nervous system. Its incidence is high (more than 50%) in the patients undergoing neurophysiological investigation, and it is considered to be more frequent in subjects with lung and breast cancers. In this study we evaluated a series of 58 women with epithelial ovarian cancer at FIGO stages I and III. The aim of the study was to assess the incidence and characteristics of peripheral nerve involvement during the course of the disease both clinically and neurophysiologically. Our results suggest that in women with epithelial ovarian cancer (1) the incidence of subclinical polyneuropathy is high; (2) sensory involvement is predominant in stage I, but motor involvement is frequent in stage III; and (3) the incidence of peripheral nerve involvement increases with progression of the cancer.     

Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopathy

In order to define diagnostic criteria for the demyelinating polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS), we compared 30 patients with idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) without a monoclonal gammopathy, with 29 patients with polyneuropathy associated with MGUS. All 59 patients fulfilled research criteria for CIDP. In the patients with MGUS, sensory symptoms and signs predominated, there was usually no cranial nerve involvement, and the neuropathy was symmetrical with a slowly progressive course. On electrophysiological examination, an abnormal median nerve sensory action potential in combination with a normal sural nerve action potential (AMNS) was not found. In idiopathic CIDP patients, a preceding infection was frequent, motor features predominated, there was often cranial nerve involvement, the neuropathy could be asymmetrical, and AMNS was frequently found. Diagnostic criteria for demyelinating polyneuropathy associated with MGUS are presented.     

小脑前下动脉梗死的临床与磁共振成像的研究

目的 研究小脑前下动脉(AICA)梗死的临床表现及磁共振成像(MRI)特征。方法 对我院1997年1月～2001年6月4年半间通过磁共振成像(MRI)确诊的19例AICA梗死患者进行临床及MRI观察。结果AICA梗死占同期急性脑梗死的1.47％,占同期小脑梗死22.1％。危险因素与一般缺血性脑卒中午相似,即高血压、高脂血症、糖尿病是其主要的危险因素。所有患者均有眩晕及小脑性共济失调的症状体征;除一名患者外均有颅神经受累,以Ⅷ、Ⅶ、v最多见。第Ⅶ颅神经受累是AIcA梗死的一个特征性改变。AICA梗死预后较好。结论 AICA梗死并非罕见,应提高对AICA梗死临床表现及MRI特征的认识,以降低临床误诊率。     

血清检测多种抗神经节苷脂抗体同时出现阳性结果临床意义分析

目的探讨血清检测多种抗神经节苷脂抗体同时出现阳性结果的临床意义及可能原因。方法回顾分析11例多种神经节苷脂抗体同时出现阳性结果患者的临床资料。结果全部11例患者均出现抗GM1-IgM抗体及抗GD1b-IgM抗体检测阳性,临床症状学研究和电生理检查均显示患者运动受累明显,而感觉受累并不显著。其中7例患者还同时出现了抗GQ1b—IgM抗体检测阳性,表现为明显的运动障碍,颅神经受累,腱反射减低以及共济失调,感觉症状不明显。结论多种抗神经节苷脂抗体检测同时出现阳性结果的患者在症状学上并没有完全表现为相应单个抗神经节苷脂抗体对应症状的组合,提示出现多种抗神经节苷脂抗体可能存在着特殊的机制。     

Sarcoidosis and the nervous system

Neurologic involvement occurs in 5 per cent of patients with sarcoidosis and is a presenting symptom in about half of those affected. The neurologic symptoms are due to a granulomatous process that mirrors that seen in the systemic form. Its diagnosis is usually established by the demonstration of sarcoidosis in other systems and supported by clinical, radiographic, and laboratory evidence. Any and all portions of the nervous system can be involved in either an acute or a chronic fashion. Early acute presentations tend to have excellent outcomes and the overall prognosis for the disease is good. The cranial nerves, especially the facial nerve, are most frequently involved and have a good prognosis. CNS involvement, usually in the form of a meningoencephalitis involving the hypothalamic region, is usually chronic and associated with poor prognosis. Peripheral nerve and muscle involvement is frequently asymptomatic. Although neuropathy or myopathy may present acutely, the usual presentation is subacute or chronic. There have been no controlled studies of therapy in neurosarcoidosis. Early implementation of corticosteroids remains the mainstay of treatment. Evidence for improvement with treatment is anecdotally reported in many cases, but progression of the disease also occurs despite therapy.     

The treatment of meningiomas in the region of the cavernous sinus

The diagnosis and treatment of meningiomas of the cavernous sinus remains a controversial part of skull base surgery practice. The most common presenting symptom headache is nonspecific. Visual loss and oculomotor disturbance are the next likely symptoms and signs. It is possible to achieve total tumor removal in about 50% of patients, but an increase in cranial nerve dysfunction occurs in nearly as many. Improvement of neurological function, except for vision, is unusual. Because of the risk of complicating cranial nerve dysfunction by surgery, focused radiation as an alternative treatment has been explored in a small number of patients. Unfortunately, volume reduction is only seen in about one-third, but risks do appear to be low and nearly 60% of patients recorded have improvement in preoperative cranial nerve deficits. With these indolent tumors, much longer follow-ups are required before the role of focused radiation can be assessed. Received: 23 December 1999     

Diffuse lower motor neuron disease in a carcinomatous meningitis

INTRODUCTION: Between 4 and 15% of solid cancers are associated with carcinomatous meningitis ant its unfavorable prognosis. The clinical presentation of neoplastic meningitis typically associates cerebral signs, cranial nerve involvement, and medullary or radicular symptoms. OBSERVATION: We report a case of a 58-year-old woman, with a history of breast cancer in remission since 8 years, who presented an acute paraparesia and a pure motor deficit of the left arm. This diffuse lower motor neuron disease was the inaugural sign of carcinomatous meningitis. CONCLUSION: The diagnosis of cacinomatous meningitis is based on medullar MRI and lumbar puncture. In same cases, clinical signs are limited to a pure diffuse lower motor neuron disease.     

脑膜癌病的临床特征及误诊原因分析

目的 分析11例脑膜癌病的临床特征及误诊原因。方法 对2002年7月-2003年12月收治的11例经脑脊液细胞学证实的脑膜癌病患的临床资料进行分析。结果 首发症状多以头痛、呕吐为主，亦可有视觉障碍、复视、眼睑闭合无力、口角歪斜以及发作性上背部疼痛，可伴脑神经和脊神经损害及脑膜刺激征；头部CT及MRI均无异常发现。脑脊液检查颅内压均不同程度增高(200-400mmH2O)，其中8例细胞数、蛋白升高，糖、氯化物下降；细胞学检查均发现癌细胞，形态与原发肿瘤相似，来源于肺癌5例、乳腺癌3例、胃癌1例、卵巢癌1例、血液系统恶性淋巴瘤1例。其中5例于发现原发肿瘤之前即有神经系统症状。11例患中6例病情恶化自动出院，2例院内死亡，3例经对症治疗症状缓解出院。结论 对以头痛、呕吐发病伴脑神经、脊神经受损，并出现脑膜刺激征和颅内压增高，头部CT及MRI无明显异常，应考虑脑膜癌病的可能，及早行全身检查寻找原发病灶，以脑脊液发现癌细胞为确诊依据。     

Malignant non-Hodgkin lymphoma presenting with Garcin's syndrome

INTRODUCTION: R Garcin described progressive unilateral cranial nerve palsy in 1926. Garcin syndrome is characterized by progressive involvement of the cranial nerves culminating in total unilateral paralysis of all cranial nerves. Carcinoma of the skull base or ENT regions is the most common etiology. CASE REPORT: A 74-year-old man developed signs involving the left Vth (V2 and V3) cranial nerve then the VIth, VIIth and VIIIth cranial nerves and finally the IXth and Xth. MRI showed involvement of these cranial nerves with gadolinium uptake and involvement of the pons at the terminal phase. Careful ENT explorations failed to reveal a cause. The lymphocyte count was elevated in the cerebrospinal fluid. The patient died one year after diagnosis and the general autopsy was normal. The neuropathological studies led to the post-mortem diagnosis of type B non-Hodgkin lymphoma. CONCLUSION: In patients with Garcin syndrome, lymphoma is a possible diagnosis when carcinoma of the ENT regions or of the skull bases are not present.