Cytomegalovirus neuropathy in acquired immunodeficiency syndrome: a clinical and pathological study.

We have observed typical cytomegalovirus cytopathology associated with multifocal inflammatory and necrotic lesions of peripheral nerve in biopsy specimens from 4 patients who developed a rapidly progressive, multifocal neuropathy late in the course of human immunodeficiency virus infection. The inflammatory infiltrates, which contained numerous polymorphonuclear cells, were associated with mixed, axonal, and demyelinative lesions of nerve fibers. One of these patients improved on treatment with DHPG (9-[2-hydroxy-l(hydroxymethyl) ethoxymethyl] guanine) and remains stable after 18 months.

Dominantly transmitted congenital indifference to pain.

Two patients from a family with dominantly inherited indifference to pain were investigated. Perception of the other sensory modalities was normal as was the remainder of the neurological examination. Electrophysiological studies and morphometric evaluation of myelinated and unmyelinated fibers of nerve biopsy specimens were normal.

[Neurologic complications in hemodialysis patients].

Various neurological complications may occur in patients under haemodialysis for end-stage chronic renal failure. Their frequency has clearly been reduced by improvements in the modalities and techniques of dialysis. Some of these complications are related to uremia and/or to the accumulation of endogenous toxic substances the nature of which has not been elucidated (e.

Molecular genetics of amyloid neuropathy in Europe.

The Portuguese type of familial amyloid polyneuropathy (FAP type I), a disabling autosomal dominant disorder with onset in early adult life, is caused by a point mutation in the transthyretin (TTR; previously known as prealbumin) gene. DNA analysis in thirteen European families (one British, two French, one Italian, one Greek, and eight Cypriot) showed that members of all those from Cyprus and Greece, and one from France, carried the FAP type I mutation. Patients from seven of these ten kindreds were not known to have a genetic disease before this study, which demonstrated the mutation in 16 of 43 clinically unaffected relatives.

[Poisoning by beta,beta'-iminodipropionitrile (IDPN) in the rat: an experimental model for elective lesions of peripheral motoneurons].

Chronic or acute intoxication of rats with beta,beta'-iminodipropionitrile induces characteristic lesions of axons of anterior horn cells. Swellings of proximal axons are associated with disorganization of the cytoskeleton which includes a disorientation of neurofilaments which are segregated in the periphery of the axon, while microtubules and mitochondria are clustered centrally. Slow axonal transport, which is markedly reduced, results in accumulation of neurofilaments in the proximal part of the peripheral motoneuron and distal atrophy.

[Acute and reversible axonal polyneuropathy in post-leptospirosis].

A 53 year old woman presented, one month after an anicteric leptospirosis, an acute, asymmetrical, sensorimotor polyneuropathy involving the lower limbs. Electrophysiological study showed evidence of severe denervation, with normal motor nerve conduction velocities, indicating an axonal degeneration. Neuro-muscular biopsy showed signs of wallerian degeneration and perivascular infiltrates of epineural vessels.

Inflammatory lesions of peripheral nerve in a patient with human T-lymphotropic virus type I--associated myelopathy.

In a patient with tropical spastic paraparesis associated with a positive titer for human T-lymphotropic virus type I, electrophysiological study detected a mixed, axonal and demyelinating, multifocal neuropathy. Perineural and perivascular infiltrates, moderate axon loss, wallerian degeneration, and demyelinating lesions of isolated fibers were present in the nerve biopsy specimen. These inflammatory lesions resembled those found in the central nervous system of patients with tropical spastic paraparesis.

Schwann cell degeneration induced by doxorubicin (adriamycin).

Doxorubicin is an anthracycline antineoplastic antibiotic that acts at the cell nucleus by intercalating between base pairs of DNA, thus inhibiting DNA-directed mRNA synthesis. Intraneural micro-injection of 0.19-0.

The peripheral neuropathy of necrotizing arteritis: a clinicopathological study.

We have reviewed the clinical and morphological data from 100 patients with necrotizing arteritis in muscle and/or in nerve samples taken by biopsy. The neuropathy occurred in the context of a multisystem disorder (Group 1) or in apparent isolation (Group 2). The average age of patients was 59 in Group 1 and 61 in Group 2.

POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions.

A rare form of plasma cell dyscrasia characterized by the various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes has been termed POEMS syndrome. The pathogenesis of the multisystemic features of this syndrome remains unclear. Herein is reported a case of POEMS syndrome with striking clinical similarities with scleroderma, and microangiopathic glomerular lesions, as well as diffuse perivascular non-amyloid deposits, which could explain certain features of the syndrome, including peripheral nerve demyelination.

Persistence of Trypanosoma cruzi antigens in the inflammatory lesions of chronically infected mice.

Different tissues and organs of mice infected with Trypanosoma cruzi trypomastigotes have been examined for the presence of parasites and parasitic antigens during both the acute and the chronic phases of infection. Specimens of skeletal and cardiac muscles, spleen, liver, brain and sciatic nerves were studied by histological and immunological methods. During the acute phase of infection, the parasites were commonly observed in these tissues.

Cellular immunity to Trypanosoma cruzi is mediated by helper T cells (CD4+).

The infection of mice with Trypanosoma cruzi has been used as an experimental model for human Chagas disease, because the murine and human infections have similar acute and chronic phases generating similar immunopathological phenomena. Histopathological studies of murine tissues showed that the inflammatory lesions were small during the acute phase and composed mainly of mononuclear cells. During the chronic phase, cellular infiltrates were clustered in large granulomata consisting of mononuclear and polynuclear neutrophil cells.

L3T4+ T cells able to mediate parasite-specific delayed-type hypersensitivity play a role in the pathology of experimental Chagas' disease.

During the chronic phase of infection with the parasite Trypanosoma cruzi, mice develop inflammatory lesions in the heart and skeletal muscles, as well as in peripheral nerves and the liver. We demonstrated the presence, in the blood of chronically infected mice, of L3T4+ T cells able to transfer a specific T. cruzi delayed-type hypersensitivity (DTH) reaction.

[Action of dihydroergotamine in severe orthostatic hypotension. 16 cases].

A cooperative study of dihydroergotamine in the treatment of severe postural hypotension was conducted in 16 patients. Postural hypotension was related to Shy-Drager syndrome in 9 and to diabetic neuropathy in one; it was idiopathic in 6 patients. With an average daily dose of 36 mg orally, the fall in systolic blood pressure when passing from supine to standing position was 39 mmHg versus 59 mmHg (mean values) before treatment.

[Acquired metabolic neuropathies (2): Kidney failure, hypothyroidism and hypoglycemia].

This is the second part of a review article on metabolic neuropathies. The frequency and severity of neuropathy due to chronic renal failure have dramatically decreased during the last ten years, as a result of improvement in management of patients with renal failure, even though the mechanism leading to development of the neuropathy is not well understood. Measurement of nerve conduction velocity does not appear to be appropriate to the detection of axonal neuropathy and follow-up of patients with neuropathy associated with chronic renal failure.

Hypotrophic and dying-back nerve fibers in Friedreich's ataxia.

Eight patients with Friedreich's ataxia showed profound reduction in the density of large myelinated fibers in sural nerve biopsies. The density of small myelinated fibers was normal, but the axonal size and myelin thickness were reduced. Demyelination, presumably secondary to axonal dysfunction, was observed in 3% of the isolated fibers.

Neuropathy associated with experimental Chagas' disease.

Mice were acutely and chronically infected with Trypanosoma cruzi and then examined histologically for the presence of lesions in the peripheral nervous system. In acutely infected animals, small lymphocytic and macrophagic infiltrates were found in the nerves in association with intracellular parasites. Little or no nerve damage was present at this stage.

Contractile performance following regression of left ventricular hypertrophy in hypertensive patients.

The left ventricular end systolic stress-end systolic dimension (ESS-ESD) relation was used to assess the effect of regression in left ventricular hypertrophy on myocardial contractility in 14 hypertensive patients (mean age 47 years) treated with guanfacine (a sympatholytic central alpha-adrenergic agonist) for 10 weeks. Echocardiography (M-mode under two-dimensional guidance) was used to determine left ventricular dimensions, posterior wall thickness (PWT) and septal thickness (ST) before and during the last week of therapy. Left ventricular mass (LVM) expressed as cross-sectional area (CSA) and meridianal wall stress at end systole were derived.