1/397. Hyperactive rhizopathy of the vagus nerve and microvascular decompression. Case report. A 37-year-old woman underwent microvascular decompression of the superior vestibular nerve for disabling positional vertigo. Immediately following the operation, she noted severe and spontaneous gagging and dysphagia. Multiple magnetic resonance images were obtained but failed to demonstrate a brainstem lesion and attempts at medical management failed. Two years later she underwent exploration of the posterior fossa. At the second operation, the vertebral artery as well as the posterior inferior cerebellar artery were noted to be compressing the vagus nerve. The vessels were mobilized and held away from the nerve with Teflon felt. The patient's symptoms resolved immediately after the second operation and she has remained symptom free. The authors hypothesize that at least one artery was shifted at the time of her first operation, or immediately thereafter, which resulted in vascular compression of the vagus nerve. To the authors' knowledge, this is the first reported case of a hyperactive gagging response treated with microvascular decompression. The case also illustrates the occurrence of a possibly iatrogenic neurovascular compression syndrome. ( info) |
2/397. Cervical foraminotomy: an effective treatment for cervical spondylotic radiculopathy. Between 1983 and 1994, posterior cervical foraminotomy as described by Frykholm was performed on 89 patients with exclusively radicular symptoms caused by cervical osteophytes. The main presenting feature was arm pain. Objective neurological signs were present in 50% of the patients. At mean postoperative follow-up of 8.6 months, 95.5% of patients reported excellent or good results, while 4.5% were not improved. No patient was rendered worse following the procedure. There were no deaths and the complication rate was 2.2%. Further surgery for recurrent root symptoms was required by 6.7% of patients. Our findings are in keeping with the good results and low complication rate of this procedure as described in other studies. Informal inquiries suggest that this procedure is not widely used, at any rate in the United Kingdom, and we present this series in order to emphasize the efficacy and safety of this procedure. ( info) |
3/397. Lessons to be learned: a case study approach. Primary hyperparathyroidism simulating an acute severe polyneuritis. The case is presented of a 65 year old lady with recent onset of neuromuscular manifestations, comprising paraparesis, areflexia and unsteady gait, along with episodes of slurring of speech and diplopia, later confirmed to be due to severe hypercalcaemia--which itself was caused by primary hyperparathyroidism. Restoration of normocalcaemia, by means of rehydration and bisphosphonate therapy, resulted in clinical improvement--whilst subsequent parathyroidectomy was followed by complete resolution of all symptoms. In order to make prompt differentiation between the neurological sequelae of hyperparathyroidism and a primary neurological disorder, a high index of suspicion is required. An urgent serum calcium assay, as part of a bone profile, is mandatory in patients who present with neurological symptoms--especially the elderly, amongst whom hyperparathyroidism is especially common. ( info) |
4/397. A large Japanese family with machado-joseph disease: clinical and genetic analysis. We report clinical and genetic studies on a large Japanese family with machado-joseph disease (MJD), in which various different clinical phenotypes were seen in the same family, i.e., cerebellar ataxia type, severe amyotrophy type, and young-onset parkinsonism type. In addition, patients with very mild symptoms (formes frustes) were encountered. The expansion of the CAG repeat at the MJD locus ranged from 64 to 71 in 7 affected and 4 presymptomatic individuals. In our family, no clear inverse correlation was noted between the length of CAG-expansion and the age of onset, or the clinical phenotypes. Hyporeflexia was a common manifestation seen in 5 patients. It has been reported that the presence of peripheral neuropathy in MJD is associated with smaller increase in the CAG repeats; findings in our family conform with this observation. ( info) |
5/397. Neurological deficit following spinal anaesthesia: MRI and CT evidence of spinal cord gas embolism. A 62-year-old diabetic woman developed permanent neurological deficits in the legs following spinal anaesthesia. MRI showed oedema in the spinal cord and a small intramedullary focus of signal void at the T10 level, with negative density at CT. Intramedullary gas bubbles have not been reported previously among the possible neurological complications of spinal anaesthesia; a combined ischaemic/embolic mechanism is hypothesised. ( info) |
| During a 15-year period, 29 children, under the age of 6 years, with acute guillain-barre syndrome were seen at our institution. A review of their charts revealed that pain was a symptom in all patients and was present on admission in 79% of cases. pain was often the most important symptom and led to misdiagnosis in 20 patients (69%). In 11 of these children, symptoms were present for more than a week before the correct diagnosis was made. The most common pain syndrome was back and lower limb pain, present in 83% of patients. Pediatricians should consider guillain-barre syndrome in their differential diagnosis when faced with a child who has lower limb pain and areflexia. ( info) |
| A 44 year old female presented with fever, muscle aches, rash and a low platelet count. IgM antibody to dengue virus was positive. Two weeks later she developed a flaccid areflexic quadriparesis. Nerve conduction studies showed a predominantly demyelitinating sensory motor polyneuropathy consistent with guillain-barre syndrome. Despite the relatively common occurrence of dengue fever, an associated polyradiculoneuropathy is distinctly uncommon. ( info) |
8/397. Cardiac sympathetic denervation in Ross syndrome demonstrated by MIBG-SPECT. We investigated cardiac sympathetic innervation by metaiodobenzylguanidine (MIBG) imaging in a patient with tonic pupils, loss of tendon reflexes, and segmental anhidrosis (Ross syndrome). Despite normal cardiovascular reflex tests, we observed a reduced global myocardial MIBG uptake as well as a regional uptake defect over the posterolateral cardiac territory indicating left ventricular peripheral sympathetic denervation. MIBG imaging seems to be a useful noninvasive diagnostic method for detection of early--possibly subclinical--cardiac autonomic impairment in Ross syndrome and provides further evidence of injury to postganglionic autonomic neurons as the underlying pathological mechanism of the disease. ( info) |
| In rare cases, miller fisher syndrome (MFS) has been known to recur. However, clinical features of recurrent MFS have not been well analyzed, and the precipitating factors relating to recurrence remain unknown. From 1981 to 1996, we examined four patients with recurrent MFS among 28 Japanese MFS patients. In the four patients, the recurrent episodes occurred after long asymptomatic intervals, ranging from 2.5 to 12.5 years. The clinical and laboratory features of recurrent episodes were similar either to those of the initial episodes or to those of the 24 non-recurrent patients. Of the two patients tested for serum IgG anti-GQ1b antibody, both were positive. Serological HLA typing showed that all recurrent patients were both HLA-Cw3 and -DR2 positive. However, out of 13 non-recurrent patients examined, six had HLA-Cw3, and four had HLA-DR2. The frequency of HLA-DR2 among the recurrent patients was significantly higher than among healthy controls (corrected P = 0.038), and was also higher than among the non-recurrent patients but not significantly. These findings suggest that recurrent MFS is clinically the same as typical MFS and that HLA-DR2 is possibly associated with recurrence. ( info) |
| Spinal extradural angiolipomas are distinct, benign, and rare lesions composed of mature lipocytes admixed with abnormal blood vessels. They account for 0.14% of all spinal axis tumors. The case described here was a 72-year-old patient presenting with a history of paraparesis, hypoesthesia under the T2 level, hyperreflexia, and urinary overflow incontinence that appeared within 7 days after the administration of a coronary vasodilator drug regimen. The spinal magnetic resonance scan showed a lipomatous mass with signal void lesions, suggesting a vascular component of the tumor. The patient improved rapidly after surgical resection of the epidural tumor and decompression of the cord. According to the present literature, the duration of neurological symptoms ranges from 1 to 180 months (mean 28 months). But this patient's neurological deterioration took place 4 days before hospitalization. We believe that this can be explained by the increased tumor blood volume caused by vasodilator drugs, which in turn exerted a pulsatile compressive effect on the cord. ( info) |