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J C Turpin

Publications and source records attributed to J C Turpin.

87 records · Page 5Linked to original sources
PubMedDate not supplied

[Adrenoleukomyeloneuropathy presenting as a mental disorder].

Troubles of cognitive functions are generally observed in the X-linked adrenoleucodystrophy. This disease can be revealed at late onset (adrenomyeloneuropathy) or contemporary of the demyelinisation in central nervous system (adrenoleucodystrophy). Cerebral variants with only psychic involvement remain uncommon, the lesions are located in the temporo-parieto-occipital areas. The diagnosis occurs by biological investigations and/or the familial history before any defect of neurological signs. Therapeutics hopes in this storage disorder lay by the earlier diagnosis. In this way, we found interest to investigate the adult forms.

Adrenoleukodystrophy↗
PubMedDate not supplied

[Assays of arylsulfatase activity in psychotic patients. Review of the literature and results of a study of 22 patients].

After a clinical and biological description of arylsulfatase A and metachromatic leucodystrophy, we present the results of a study of leucocyte arylsulfatase A in a population of 22 adult psychotic inpatients. The patients, clinically defined, filled the criteria of DSM III of schizophrenic disorders. All of them were treated by neuroleptics. None of these 22 patients showed a level of arylsulfatase A different from the range of 27 healthy adult controls. These results differ from those of most precedent studies of arylsulfatase A in "psychiatric populations". Some explanations of this difference are suggested. The concept of "purely psychiatric" form of adult metachromatic leucodystrophy is discussed.

Adult↗
PubMedDate not supplied

[Myotonic dystrophy severity (Steinert's disease) (author's transl)].

Four family cases of Steinert, myodystrophy are reported. The severity of the early onset form is specially noted. This form presents pulmonary complications and mental deficit. The influence of the myodystrophy on the pregnancy is discussed and an explanation of the disturbance of the oesophaguspharynx motility is presented. Physiopathogenesis problems of the Steinert's disease are noted.

Adolescent↗
PubMedDate not supplied

[Interest of ganglioside analysis in the neuroblastoma (author's transl)].

As suggested by Hakomori, one of the mechanisms to explain the abnormal proliferation of tumor cells by loss of contact inhibition could be a modification of membrane gangliosides. We report here a case of neuroblastoma with opsomyoclonia in which the ganglioside profile is abnormal, with a high level of GP3 and GM2, and of other gangliosides which are not present in normal child and adult brains.

Brain Neoplasms↗
PubMedDate not supplied

[Present data of Fahr's syndrome. Report of two cases of idiopathic strio-pallido-dentate calcifications revealed by computerized tomography (author's transl)].

The authors report two computed tomographic observations of Fahr's disease without disturbances in calcium-phosphorus metabolism; the first case is a by chance discovery and the second one is characterized by a cerebellar tremor. From the literature data they discuss successively clinical, radiological and computed tomographic, neuropathological, aetiopathogenetic and therapeutic aspects actually presented by strio-pallido-dentate calcifications (SPD) or Fahr's disease.

Adult↗
PubMedDate not supplied

[Carbon monoxide poisoning of the fetus. Apropos of a clinico-pathological case].

Anatomical and clinical observations are described in a case of carbon monoxide poisoning which caused the death of the baby rapidly. The anatomical lesions are comparable to those observed in 8 other cases reported in the literature. The physiopathological mechanism involved is discussed in relation to previously reported clinical, toxicological, and experimental data.

Adult↗