Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A five-year-old girl developed neurogenic muscular atrophy during infancy. On the basis of clinical findings and a muscle biopsy study a diagnosis of infantile spinal muscular atrophy, benign type II, was entertained. As the disease progressed, involvement of sensory pathways was detected by electrophysiological studies. Examination of a sural nerve specimen suggested a chronic polyneuropathy rather than spinal muscular atrophy. These findings were confirmed at autopsy by demonstrating severe loss of myelinated axons in ventral and dorsal spinal roots, peripheral nerves and dorsal columns of the spinal cord. In addition, islands of astroglial fibers were found in ventral and dorsal spinal roots, regarded as a secondary reaction to the breakdown of myelinated axons. This unusual scarring process seems to result from nerve fiber loss during the perinatal period, since radicular glial scar tissue is not known to occur in the spinal muscular atrophies of later onset. Examination of the sensory nervous system in patients with Werdnig-Hoffmann disease appears mandatory to clarify the precise disease entity leading to infantile neurogenic muscular atrophy.
 ...
PMID:Peripheral motor and sensory neuropathy of early childhood, simulating Werdnig-Hoffmann disease. 18 71

Neurologic complications may appear before the primary disease, alcoholism, is recognized. The common syndromes are polyneuropathy, the withdrawal syndrome and the combination of Wernicke's encephalopathy and Korsakoff's psychosis. Other conspicuous clinical pictures include ataxia of cerebellar origin, convulsions, acute hallucinosis, myopathy and coma. Rarer disorders are Marchiafava-Bignami disease and central pontine myelinolysis.
 ...
PMID:The neurologic complications of alcoholism. 18 91

Sural nerve biopsies of 5 children of patients with familial amyloid polyneuropathy were studied by electronmicroscopy. The subjects were 14-17 years old and were in normal health. Neurological examination was negative. In none of the sural nerve specimens were there any amyloid deposits. In Case 4 no fine structural changes were detected. In the remaining 4 cases, there were frequent deposits of glycogen and clusters of multimembranous bodies in the Schwann-cell crescents of large myelinated fibres which presented infoldings of the myelin sheath, irregular myelin lamellation and a great number of Schmidt-Lanterman incisures. In one nerve fasciculus of Case 5 the myelin sheaths of some large myelinated fibres were extremely thickened; the axoplasm exhibited dilated vesicles and disordered microfilaments. These findings were taken as evidence of the occurrence of nerve fibre lesions at the pre-symptomatic stage of the disease, which seem to precede the appearance of amyloid deposition. The lesions affected primarily the Schwann cell and myelin sheath, and spared the unmyelinated fibres.
 ...
PMID:Peripheral nerve fibre changes in asymptomatic children of patients with familial amyloid polyneuropathy. 18 58

The authors report about an own case of recurrent sporadic hypertrophic polyneuropathy and describe the clinical course and histologic picture with reference to the literature. The disease is characterized by recurrences of subacutely occurring polyradiculoneuropathy and sequent nearly complete remission. Clinical examination discloses preferentially symmetrically and distally occurring motor paresis while sensibility in most cases is less affected. The peripheral nerves may be enlarged after a few relapses and frequently painful to pressure during the bout. Excessive increase in CSF proteins is found only during the bout. Motor nerve conduction velocity is considerably reduced. Histological pictures typically present an onion bulb formation of the Schwann cells with marked proliferation of connective tissue. There frequently younger individuals are involved; the relation female to male is 3:1. Differentiation has to be made concerning hereditary and symptomatic forms of hypertrophic polyneuropathy. Etiological factors of the disease are discussed.
 ...
PMID:[Sporadic recurrent hypertrophic polyneuropathy. Clinical-histological contributions on differential diagnosis]. 18 90

During clinical and electromyographical examination of one patient with diabetic and one with alcoholic polyneuropathy we found an especially severe lesion of the ulnar nerve at the elbow on one side. As the polyneuropathy generally affects the nerves symmetrically we presumed in addition some other most probably ulnar nerve compression syndrome at the elbow. Both patients were operated and the nerves were transposed (Prof. NIGST). The local finding during the operation, the improvement of the paresis of ulnar nerve and the worsening of the polyneuropathy--found simultaneously at control after 2 and 4 years--confirmed our supposition. Should in cases of polyneuropathy an especially severely damaged nerve be found, one ought to think of the possibility of an additional mechanical lesion and treat the patient accordingly.
 ...
PMID:[Decompression of individual nerves in anatomical strictures in polyneuropathies]. 18 65

The authors report a case of polyneuropathy of lower extremities developing in carcinomatosis of spinal meninges in a patient after gastrectomy for mucus-producing carcinoma.
 ...
PMID:[Polyneuropathy syndrome in a case of spinal meningeal carcinomatosis]. 18 23

Peripheral neuropathy occurred in a 32-year-old man who was receiving dapsone (Aviosulfon) for dermatitis herpetiformis. Neurological examination showed polyneuropathy that involved the median and ulnar nerves, with a lesser involvement of other peripheral nerves. After treatment with dapsone was discontinued, the neuropathy reversed itself, with practically complete return of function of all nerves involved.
 ...
PMID:Dapsone-induced peripheral neuropathy. 18 85

Among 50 patients who suffered from chronic renal insufficiency and who were on a chronic hemodialysis, 25 demonstrated uremic polyneuropathy with a different degree of expressiveness which depended upon the duration azotemia. A histological study of 13 postmortem cases detected a microfocal demyelinization in the peripheral nerves, and in cases of expressed pathology there were changes in the axon cast of the peripheral nerves and neurogenic muscular atrophy. There were no direct correlations between the development of polyneuropathy and the amount of hemodialysis sessions.
 ...
PMID:[Polyneuropathy in uremic patients in chronic hemodialysis (clinico-morphologic findings]. 18 35

A further case of giant axonal neuropathy is described. The diagnosis was made by sural nerve biopsy in a boy presenting signs of a progressive spinocerebellar syndrome with polyneuropathy. Ultrastructurally a severe abnormality of this peripheral nerve, with loss of thick myelinated nerve fibers and the presence of giant axonal swellings was evident. The axonal swellings appeared to be caused by an accumulation of filaments which were also present in Schwann cells, as well as in endothelial and perineurial cells, justifying the term of "generalized microfibrillar hyperplasia". It is most remarkable that the patient's hair was not unusual, in contrast to the previously reported cases who had "kinky hair".
 ...
PMID:Giant axonal neuropathy. Report of a case with normal hair. 19 Mar 56

Two sporadic cases of amyloid polyneuropathy with clinical features corresponding to the Portuguese type of this disease were studied. Histological examination of sural nerve demonstrated a marked loss of myelinated and unmyelinated fibres in the case 1 due to axonal degeneration, high content of fibers with segmental demyelination and the occurrence of several enlarged axons filled with the 10 nm filaments (so-called giant axons). In the case 2 there was total loss of unmyelinated axons and myelinated fibers were nearly completely lacking. In the development of changes in the myelinated fibers their direct compression by amyloid deposits seems to play an important role. It leads to the appearance of both axonal degeneration and segmental demyelination. The latter seems to be due to local compression and it may involved many fibers. In the light of observations reported by other authors the mechanism of changes developing in unmuelinated fibers is explained by the presence of changes in the cells of posterior root ganglia, however the question whether some abnormalities seen in unmyelinated axons could not be related to the pressure exerted by amyloid deposits directly to these fibers, remains open.
 ...
PMID:Some histological aspects of amyloid polyneuropathy. 19 36


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>