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Modern techniques have defined the hereditary motor and sensory neuropathies (HMSN) as a genetically heterogeneous group of disorders. This includes a rare variant with X-linked dominant inheritance. We have traced this disorder through 6 generations of a large Canadian kindred; neurological and electrophysiological examinations were performed in 57 family(More)
Thirty patients with definite or probable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of chronic progressive (16 patients) or relapsing (14 patients) course were randomly assigned to receive intravenous immunoglobulin (IvIg) 0.4 g per kg body weight or a placebo treatment on 5 consecutive days in a double-blind, cross-over trial.(More)
When multiple sclerosis (MS) presents late in life (after the age of 50), it frequently gives diagnostic difficulty. In a large MS clinic population (N = 838), 9.4% of the patients had late onset of MS. Slow deterioration of motor function characterized the initial symptoms and subsequent course of this group of older patients. Progression of disability was(More)
Eighteen patients with definite, untreated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of chronic progressive (nine patients) or relapsing course (nine patients) were randomized prospectively to receive 10 plasma-exchange (PE) or sham plasma-exchange (SPE) treatments over 4 weeks in a double-blind trial. After a wash-out period of 5(More)
Five patients with a clinical diagnosis of acute Guillain-Barré polyneuropathy (GBP) had electrically inexcitable motor nerves. All were quadriplegic. One patient died and 3 of the 4 survivors showed poor recovery. Autopsy studies on the patient who died showed severe axonal degeneration in nerve roots and distal nerves without inflammation or(More)
The two main electrophysiological abnormalities seen in the first two weeks of the acute Guillain-Barré syndrome (GBS) were conduction block and, in some patients, low maximum M response amplitudes. In this study period, maximum motor and sensory conduction velocities were often in the normal range and temporal dispersion was relatively less common. The(More)
OBJECTIVE To assess the efficacy of carotid endarterectomy for stroke prevention in asymptomatic and symptomatic patients with internal carotid artery stenosis. Additional clinical scenarios, such as use of endarterectomy combined with cardiac surgery, are also reviewed. METHODS The authors selected nine important clinical questions. A systematic search(More)
We describe the clinical and pathologic features of experimental allergic neuritis in Lewis rats inoculated with varying doses of myelin, ranging from 0.5 to 20 mg. The clinical scores were assessed daily. On days 18 and 19 or 28 postimmunization, the rats were perfused with fixative and samples of cervical and lumbar roots and sciatic nerves were processed(More)
Four cases of severe acute Guillain-Barré syndrome (GBS) characterized by severe axonal degeneration are presented. All had electrically inexcitable motor nerves as early as 4 days after onset. The disease was rapid in onset and the residual disability was severe. Two different types of pathology were seen. Nerve biopsies in 3 cases showed severe axonal(More)