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Cortical somatosensory evoked potentials (SEPs) were recorded from a man with a severe neuropathy without touch and proprioception below the neck. Peripheral neurophysiological tests showed a complete large myelinated fibre sensory neuropathy. Sensory threshold to electrical stimulation of the median nerve was 15 mA (normal 2-4 mA). With a stimulus of 39(More)
BACKGROUND An intravenous rather than oral course of methylprednisolone is often prescribed for treating acute relapses in multiple sclerosis (MS) despite the lack of evidence to support this route of administration. Our double-blind placebo-controlled randomised trial was designed to compare the efficacy of commonly used intravenous and oral steroid(More)
The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally(More)
The results from experiments in various modalities of evoked potentials are described in a subject with a complete large peripheral neuropathy below the neck. He has no tactile or position sensitivity below that level, but has retained fatigue, pain, and temperature sensation. Percutaneous electrical stimulation of peripheral nerves led to scalp recorded(More)
We investigated the effect of oral and intravenous methylprednisolone treatment on subsequent relapse rate in patients with multiple sclerosis. Following a double blind trial designed to compare the effect of oral and intravenous methylprednisolone treatment on promoting recovery from acute relapses of multiple sclerosis, 80 patients were followed for two(More)
Transmission of Creutzfeldt-Jakob disease (CJD) from cadaveric growth hormone injections had previously been reported in 7 cases, including one from Britain. As a result, the treatment was abandoned in 1985 and superceded by safer recombinant DNA growth hormone injections. Recent reports now record the number of cases worldwide as 23, but with the(More)
A patient had transient memory loss for close family members. She could not even recognise their names as familiar. Her everyday memory was relatively preserved and she retained a clear recollection of the episode. Standard and sleep deprived EEG showed a mild abnormality of the left temporal lobe. Neuropsychological testing found evidence for a mild verbal(More)
BACKGROUND Charcot Marie Tooth (CMT) disease is a heterogeneous group of inherited peripheral motor and sensory neuropathies. CMT4H is an early onset autosomal recessive demyelinating neuropathy. The locus responsible for CMT4H was assigned to chromosome 12p11.21-q13.11 by homozygosity mapping and mutations in the Frabin gene (FGD4 Rho GDP/GTP exchange(More)
The dermatomal somatosensory evoked potential from the lumbo-sacral dermatomes was recorded from 21 patients with radiographically and surgically (20) proven lumbo-sacral root compression due to prolapsed intervertebral disc or canal stenosis. The potential was abnormal in 19 of the 20 surgically proven cases. The dermatomal somatosensory evoked potential(More)