Erythromelalgia: association with hereditary sensory neuropathy and response to amitriptyline.
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Biomedical subjects
Publications and source records attributed to S Herskovitz.
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Paroxysmal headaches often occur in benign headache disorders such as episodic cluster headache, chronic paroxysmal hemicrania (CPH) and episodic paroxysmal hemicrania. We report 2 patients with paroxysmal headaches occurring in association with cerebrovascular disease. The first patient had paroxysmal headaches from an arteriovenous malformation which resolved following embolization. In the second patient, headache followed a cerebral infarction and responded to treatment with indomethacin. We suggest that vascular disease may cause paroxysmal headaches resembling CPH. Patients with an atypical presentation of CPH warrant a neuroimaging procedure.
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We describe two patients with acquired immunodeficiency syndrome (AIDS) who developed classic thalamic syndrome (TS) due to Toxoplasma abscesses in the thalamic region. Treatment with amitriptyline provided substantial relief in both patients. Postmortem examination in one case revealed a lesion in the internal capsule and thalamic reticular nucleus. These observations indicate that (1) TS can result from an isolated lesion in the internal capsule and reticular nucleus of the thalamus, (2) cerebral abscess can cause classic TS, (3) central pain can be added to the many pain syndromes that afflict AIDS patients, and (4) an analgesic response to amitriptyline is possible in these patients.
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We examined the serial CTs and clinical courses of five patients with neuro-Behçet's disease and reviewed ten previously reported cases, all with focal CT abnormalities. The CT lesions were in the brainstem (8 patients), basal ganglia (7), thalamus (4), or hemispheric white matter (7). Of the 13 patients who received contrast, nine had lesions that showed enhancement. In five, lesions were visualized with contrast that were not apparent without it. The CT lesions were usually accompanied by corresponding clinical deficits, although in some patients deficits were more extensive than the CT predicted. In nine patients, contrast enhancement decreased or disappeared over days to weeks, often with associated clinical improvement. In eight patients followed serially, CT abnormalities resolved completely or substantially. Based on these cases, we conclude that (1) CT abnormalities of gray and white matter occur commonly in neuro-Behçet's disease with focal deficits, and help support the diagnosis; (2) CT abnormalities, particularly contrast enhancement, correlate well with the activity of parenchymal disease; and (3) the concomitant improvement of clinical and CT abnormalities with resolution of contrast enhancement suggests that partially reversible inflammatory changes associated with breakdown of the blood-brain barrier may produce some of the neurologic and radiographic deficits in this illness.
INTRODUCTION: Management of patients with radiculopathy involves estimating the degree of physiologic and anatomic injury, and weighing that to predict the likely clinical course. OBJECTIVE: To determine whether low distal peroneal and tibial CMAP amplitudes correlate with weakness and fibrillations of functionally relevant muscles in L5/S1 radiculopathy (LSR). METHODS: We reviewed clinical and electrophysiologic data in 66 consecutive patients with LSR. RESULTS: A significantly greater number of patients with low peroneal CMAP amplitudes had weakness of L5 (p = 0.025) and S1 innervated leg muscles (p < 0.001). Low tibial CMAP amplitudes were also associated with weakness of S1 innervated muscles (p < 0.038). The association of low peroneal CMAP amplitudes with weakness persisted when weakness of at least 3 muscles was considered in the analysis for L5 (p < 0.0001) and S1 (p = 0.014) innervated muscles. CONCLUSIONS: Low peroneal and tibial CMAP amplitudes may serve as surrogate measures for segmental weakness of functionally relevant muscles in LSR.