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Three cases of neuropathy with monoclonal gammopathy and amyloid deposits in peripheral nerves are described. They appeared to present a benign gammopathy because of the duration of the neuropathy in the absence of any clinical or biological sign of myeloma or macroglobulinaemia. The pathological abnormality found in the sural nerves of the three patients was characterised by a marked loss of myelinated and unmyelinated nerve fibres because of active axonal degeneration with Wallerian degeneration. The most striking feature in all three cases was the finding of deposits identified as an accumulation of microfibrils.

Postanoxic myoclonus was first accepted as being related to a dysfunction of the ventrolateral thalamic nucleus. Several stereotaxic studies have invalidated this hypothesis. The neurochemical approach, in particular the measure of 5-hydroxyindolacetic acid in the cerebrospinal fluid, has opened new theoretical and therapeutic possibilities involving serotoninergic pathways. A typical case is presented who improved markedly under a combined therapy with 5-hydroxytryptophan and a decarboxylase inhibitor. A review of the pathogenesis and therapeutic approach to postanoxic myoclonus is presented.

A case of the unusual clinical condition of cerebral coenurosis, the unique parasitic disease that is localized only in the CNS, is reported. The patient had repeated attacks of transient hemiparesis due to intracranial arteritis which was demonstrated by several angiographic studies. The CSF showed a discreet lymphocytosis and increased immunoglobulins. This inflammatory reaction which preceeded the manifestations of the intracranial mass can be explained by the parasitic foreign bodies, the liberation of somatic and metabolic parasitic toxins and the immunological reaction of the organism. The arteritis of the basal intracranial vessels was probably secondary to the inflammatory reaction of the leptomeninges. In presence of the rare association of meningovascular disease and an expanding intracranial lesion a parasitic condition due to a larval stage of Cestodes (Hydatidosis, Cysticercosis, Coenurosis) should be always considered.

Electrophysiological, histological and ultrastructural studies on two patients (one family) with familial neuropathy and a liability to pressure palsies are reported. There was slowing of nerve conduction velocities and increase of distal latencies of clinically affected and clinically non-affected nerves. These alterations were confirmed by biopsy of the sural nerive which had the characterized signs of the disease. Pathogenetically there seems to be an endogeneous metabolic defect of the Schwann cell to synthetize a normal myelin sheath, which is responsible for the particular susceptibility to mechanical damage.