Encéphalopathie infantile familiale avec nécrose bilatérale et symétrique des corps striés

Bargeton-Farkas, E.; Cochard, A.M.; Brissaud, H. E.; Robain, O.; Balle, J.C. Le

doi:10.1016/0022-510x(64)90112-1

Cited by 24 publications

(6 citation statements)

References 15 publications

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“…In this group, the temporal relationship between an acute febrile illness and the onset of neurological signs is consistent with a parainfectious encephalitis, as is the presence of lymphocytic meningitis in two of our patients and in the two previously reported children [4, 71. Mumps virus may have been the causal agent in two cases (Patient 3 and one case reported by Mathieson and Olszewski [7]), although in our Patient 3 a second, undiagnosed febrile episode immediately preceded the neurological disorder. N o cultural or serological evidence of any other viral infection could be obtained.…”

Section: Discussionmentioning

confidence: 87%

“…Both patients died as a result of intercurrent infection or nutritional problems after courses of eight and nine weeks, respectively. Central pontine myelinolysis was found at necropsy in one of them [ 7 ] , but this was probably a secondary event related to the nutritional complications [lo]. We suggest that these two cases as well as our own three patients represent an unusual acute neurological disorder of possible infectious origin.…”

Section: Discussionmentioning

confidence: 91%

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Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Goutières

Aicardi

1982

Annals of Neurology

110

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Three children ranging in age from 1 to 4 1/2 years suffered an acute illness with disturbance of consciousness followed by motor rigidity, loss of spontaneous movements and language, axial hypotonia, and a stereotyped response to any kind of stimulus. In two cases, images on computerized tomography were consistent with necrosis of the lenticular and caudate nuclei. The acute onset was followed by later improvement and stabilization. One child died, probably not as a direct consequence of the neurological disorder, autopsy showed bilateral necrosis of the putamina. We suggest that these three patients, together with two previously reported cases, constitute a clinically and radiologically recognizable subgroup among the heterogeneous disorder that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 91%

Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Goutières

Aicardi

1982

Annals of Neurology

110

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“…[5][6][7][8][9][10][11] The patients in our series, which is the largest reported so far, had similar initial clinical manifestations-i.e., developmental arrest and in some cases regression of motor and cognitive skills similar to the earlier cases-but there were differences in the extrapyramidal and ocular manifestations. [5][6][7][8][9][10][11] The patients in our series, which is the largest reported so far, had similar initial clinical manifestations-i.e., developmental arrest and in some cases regression of motor and cognitive skills similar to the earlier cases-but there were differences in the extrapyramidal and ocular manifestations.…”

mentioning

confidence: 99%

Familial infantile bilateral striatal necrosis

Straussberg¹,

Shorer²,

Weitz³

et al. 2002

Neurology

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“…Failure to find any damage is perhaps due to the inadequate sampling of nerves which this has entailed. In only 5 cases (Tuthill, 1960;Bargeton-Farkas et al, 1964); Eiben, Dooley, and Stowe, 1965;Yashon and Jane, 1967;and Worsley et al, 1965-Case 1) have there been lesions in the mamillary bodies; they were specifically described as 'normal' or 'unaffected' in all others, including the present series.…”

Section: Discussionmentioning

confidence: 48%

Necrotizing Encephalomyelopathy of Leigh: Neuropathological Findings in 8 Cases

Dayan¹,

Ockenden²,

Crome³

1970

Archives of Disease in Childhood

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. Necrotizing encephalomyelopathy of Leigh: neuropathological findings in 8 cases. Eight patients with necrotizing encephalomyelopathy are reported.Lesions were found only in the central nervous system. The principal sites affected were the caudate nuclei, putamina, periaqueductal tissues, tegmentum, and structures in the floor of the IVth ventricle. Lesions were also found in the centrum ovale, cerebellum, and the grey and white matter in the spinal cord. The histological findings were of spongy rarefaction, scarring, and excessive vascularity.The pattern of inheritance of necrotizing encephalomyelopathy suggests that it is due to an autosomal recessive gene. Though the disease closely resembles the encephalopathy of thiamine deficiency, morphological and biochemical evidence suggests that this is not a direct aetiological factor. It is possible that the condition is due to a disorder of lipoic acid metabolism, perhaps at a step near the thiamine-dependent stage of carbohydrate metabolism.

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Encéphalopathie infantile familiale avec nécrose bilatérale et symétrique des corps striés

Cited by 24 publications

References 15 publications

Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Familial infantile bilateral striatal necrosis

Necrotizing Encephalomyelopathy of Leigh: Neuropathological Findings in 8 Cases

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