1/23. 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.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/23. 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.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/23. Friedreich's ataxia presenting as adult-onset spastic paraparesis.We have studied a man with an atypical form of Friedreich's ataxia (FRDA), who presented at age 26 years with a 2-year history of unsteadiness and clumsiness. The predominant feature of his initial neurological examination was a spastic paraparesis, along with a mild distal weakness and hyperreflexia of the upper limbs. He also displayed limb ataxia. Frataxin GAA repeat sizes were 1,040/690. This unusual FRDA presentation is not dissimilar to that of Acadian spastic ataxia.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/23. Persistent paraneoplastic neurologic syndrome after successful therapy of Hodgkin's disease.Paraneoplastic neurologic syndromes may develop in Hodgkin's disease (HD). We describe three young female patients with neurological disorders, not explained by other causes, preceding diagnosis or relapse of HD. The lack of response of the paraneoplastic syndrome to successful treatment of HD among our three patients emphasizes the poor prognosis of longstanding paraneoplastic neurologic symptoms in HD.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/23. Leptomeningeal signet-ring cell carcinomatosis presenting with ophthalmoplegia, areflexia and ataxia.We report a very rare case of occult leptomeningeal carcinomatosis (LC) in whom repeated cytological examination did not show malignant cells in cerebrospinal fluid (CSF) and the primary focus was not discovered by extensive survey. The patient presented with ophthalmoplegia, ataxia and areflexia mimicking miller fisher syndrome (MFS) at the initial stage, and later, the clinical profile and laboratory findings including CSF examination simulated tuberculous meningitis. Postmortem autopsy disclosed metastatic signet-ring cell carcinoma infiltrating into cranial nerves and leptomeninges. We would like to emphasize that LC sometimes shows symptoms and signs similar to MFS or tuberculous meningitis.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/23. Sudden deafness and anterior inferior cerebellar artery infarction.BACKGROUND AND PURPOSE: Acute ischemic stroke in the distribution of the anterior inferior cerebellar artery (AICA) is known to be associated with vertigo, nystagmus, facial weakness, and gait ataxia. Few reports have carefully examined the deafness associated with the AICA infarction. Furthermore, previous neurological reports have not emphasized the inner ear as a localization of sudden deafness. The aim of this study was to investigate the incidence of deafness associated with the AICA infarction and the sites predominantly involved in deafness. methods: Over 2 years, we prospectively identified 12 consecutive patients with unilateral AICA infarction diagnosed by brain MRI. Pure-tone audiogram, speech discrimination testing, stapedial reflex testing, and auditory brainstem response were performed to localize the site of lesion in the auditory pathways. electronystagmography was also performed to evaluate the function of the vestibular system. RESULTS: The most common affected site on brain MRI was the middle cerebellar peduncle (n=11). Four patients had vertigo and/or acute auditory symptoms such as hearing loss or tinnitus as an isolated manifestation from 1 day to 2 months before infarction. Audiological testings confirmed sensorineural hearing loss in 11 patients (92%), predominantly cochlear in 6 patients, retrocochlear in 1 patient, and combined on the affected side cochlear and retrocochlear in 4 patients. electronystagmography demonstrated no response to caloric stimulation in 10 patients (83%). CONCLUSIONS: In our series, sudden deafness was an important sign for the diagnosis of AICA infarction. Audiological examinations suggest that sudden deafness in AICA infarction is usually due to dysfunction of the cochlea resulting from ischemia to the inner ear.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/23. Aicardi-Goutieres syndrome: clinical and neuroradiological findings of 10 new cases.AIM: To describe the clinical and neuroimaging findings in new cases with Aicardi-Goutieres syndrome (AGS) from egypt. methods: Ten patients with progressive encephalopathy, bilateral calcification of the basal ganglia and spastic quadriplegia were described. Feeding difficulties, irritability, unexplained episodic fever and acrocyanosis were also observed. They were diagnosed as AGS after excluding possible non-genetic causes (especially TORCH) and because of the high interferon-alpha (IFN-alpha) level in cerebrospinal fluid (CSF) in two children who underwent this specific investigation. RESULTS: Six patients had postnatal microcephaly. putamen was by far the most common site of calcification (nine cases) inside the basal ganglia. Calcifications were extended to the white matter, periventricular and cerebellum in three cases. brain atrophy and/or white matter demyelination were evident in most of the cases. Further, hypogenesis of corpus callosum was detected in two cases; one of them had in addition cerebellar hypoplasia, atrial septal defect (ASD) and horseshoe kidney. To the best of our knowledge, the association of these congenital abnormalities has not been reported before in AGS. Eight families were consanguineous. CONCLUSION: This paper presents variability in both age of onset, clinical picture and neuroimaging findings even in the same family, comprising new congenital abnormalities associated with AGS and subsequently expanding the spectrum of heterogeneity. The observation of familial cases and both affected males and females emphasized the major role of the single gene inheritance.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/23. Diffuse large B-cell lymphoma presenting as a sacral tumor. Report of two cases.Primary lymphomas of the sacrum are rare tumors, reported only in a few cases in the literature. The authors describe two patients with diffuse large B-cell lymphomas presenting as a sacral tumor. In the first case a 52-year-old man presented with progressive back pain, bilateral radicular pain, and saddle block anesthesia secondary to a lytic, expansile soft-tissue mass. The mass arose from the sacrum and eroded through the right S-1 to S-4 foramina and extended into the epidural space of the spinal canal. On magnetic resonance imaging, the sacral mass enhanced homogeneously with Gd. In the second case a 64-year-old man presented with left-sided radicular pain, paresthesias, and progressive weakness due to a lytic soft-tissue mass in the left sacral ala extending into the left L-5 and S-1 foramina. Metastatic workup in each patient demonstrated unremarkable findings. In both cases, an open biopsy procedure was performed after nondiagnostic examination of needle biopsy samples. Histopathological examination showed evidence consistent with diffuse large B-cell lymphoma in both patients. In the first case the disease was classified as Stage IAE, and the patient subsequently underwent four cycles of cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP)- and rituximab-based chemotherapy followed by consolidation radiotherapy. In the second case the disease was also classified as Stage IAE, and the patient underwent CHOP-based chemotherapy and consolidation radiotherapy. In both cases radiography demonstrated a decrease in size of the sacral lymphomas. The authors review the clinical, radiological, and histological features of sacral lymphomas. lymphoma should be considered in the differential diagnosis of sacral tumors.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/23. Segmental conduction abnormalities and myelin thickenings in Val102/fs null mutation of MPZ gene.The authors report in patients with Val102/fs null mutation a possibly age dependent variability of clinical and electrophysiologic phenotype, segmental conduction abnormalities mainly in ulnar nerves at the elbow, and excessive myelin foldings and thickenings. The authors hypothesize that myelin thickenings at the paranodal region, in concurrence with compression at usual entrapment sites or minor repetitive trauma, may induce segmental conduction abnormalities.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/23. Hyperekplexia and sudden neonatal death.Fifteen patients with hyperekplexia were identified in 3 families; diagnostic clinical characteristics were defined which allowed for early recognition and treatment. During the first 24 hours of life, spontaneous apnea and sluggish feeding effort were observed. After the first 24 hours, surviving infants exhibited the hyperekplexic startle response to nose tapping. This startle response is characterized by sudden muscular rigidity, feeding-induced oropharyngeal incoordination, and poor air exchange often with apnea, persisting with repetitive nose tapping. Untreated infants experienced recurring apnea until 1 year of age. Three of 15 patients died unexpectedly during the neonatal period. patients treated with clonazepam (0.1-0.2 mg/kg/day) had no serious apneic episodes and startle reflexes were diminished. The pathophysiologic mechanism for hyperekplexia remains obscure. Electroencephalographic studies were consistently normal. The response to and tolerance of benzodiazepines are striking in newborns and infants and suggest an aberrant central nervous system reflex as the etiology; therefore, hyperekplexia should be considered in the evaluation of neonates and infants with apnea, aspiration pneumonia, episodic muscular rigidity, hyperexcitability, and near-miss sudden infant death syndrome. The need for immediate monitoring of at-risk infants, observation for signs of hyperekplexia, and initiation of clonazepam in these patients are emphasized. Hyperekplexic startle response to nose tapping should be included in the routine examination of all newborns.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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