The pathology of a radial nerve biopsy in leprosy: light and electron microscopy.
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Biomedical subjects
Publications and source records attributed to P Kahn.
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A clinical review and histopathological study of three cases of Anderson-Fabry disease is presented and pathological changes in the central and peripheral nervous systems are reported, in some sites for the first time. These are telangiectatic changes in vessels of the sympathetic ganglia in the vertebral trunk; storage of glycolipid in pigmented cells of the substantia nigra and in anterior horn cells; and degeneration of nerve fibres in the dorsal root entry zone and substantia gelatinosa of the spinal cord. The histopathological findings suggest that in this disease pain is due to involvement of dorsal root ganglion cells with associated axonal degeneration of the small fibres in pathways subserving pain.
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A case is described in which the clinical and pathological features of amyotrophic lateral sclerosis (ALS) occurred in a patient with cysticercosis of the central nervous system and associated pachymeningitis of the cervical spinal cord. This is an extremely rare finding. The patient also presented the two symptom complexes more commonly encountered in this condition: focal and generalized epilepsy, and signs of obstructive hydrocephalus. The literature relevant to this case, and to cysticercosis in Great Britain, is briefly reviewed. The case described adds to the concept of motor neurone disease as a syndrome rather than a disease entity of undetermined aetiology.
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