Search PubMed⌕ Search

Biomedical subjects

J H French

Publications and source records attributed to J H French.

At least 37 records · Page 2Linked to original sources
PubMed1979

Thalamic degeneration in X-chromosome--linked copper malabsorption.

Thalamic degeneration was present in 5 autopsied cases of X-chromosome-linked copper malabsorption (X-cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuronal depopulation. The thalamic nuclei that send axons to the agranular cortices were less often and less severely involved. The thalamic afferent system was intact except for degeneration of the red nucleus. Cerebral cortical lesions varied from case to case and usually were less marked than thalamic neuronal changes.

Adolescent↗
PubMed1979

Degeneration of the cerebellar system in X-chromosome-linked copper malabsorption.

Examination of the cerebellar system of 5 autopsied patients with X-chromosome-linked copper malabsorption led to the discovery that among cerebellar afferent systems, only the dorsal spinocerebellar tract showed consistent degenerative changes. Cerebellar cortical lesions comprised granule cell loss and Purkinje cell abnormalities that included nuclear and cytoplasmic degenerative changes with cell loss and deficient dendritic arborization as well as the presence of somatic sprouts. Perisomatic baskets were often absent in the area of sprouting Purkinje cells. Cerebellar cortical topographical analysis revealed relative sparing of the caudal portion of the cerebellum. The nodulus was intact in all patients. The dentate nucleus and the superior cerebellar peduncle were preserved. The red nucleus was consistently degenerated.

Adolescent↗
PubMed1977

Fine structure of the cerebellar cortex in Menkes Kinky-hair disease. X-chromosome-linked copper malabsorption.

The cerebellar cortices in two patients with X-chromosome-linked copper malabsorption (Menkes kinky-hair disease) were examined with both the light and electron microscope. Somatic sprouts and grotesque dendritic arborization were among the obvious light microscopical changes. At the fine structural level, the Purkinje cells were characterized by the presence of somatic spines although the surviving granule cells had already descended. Most spines were parts of synaptic complexes, but some were unattached to any presynaptic element. Similar spines were also observed on dendritic trunks. Astrocytic gliosis and nonspecific endothelial cell changes were noted.

Axons↗
PubMed1977

The electroencephalogram in childhood basilar artery migraine.

In two pediatric patients with the clinical picture of basilar artery migraine, transient electroencephalographic disturbances were seen. Both children manifested posterior rhythmic delta activity in close temporal proximity to their attacks. Resolution of these abnormalities was documented by serial electroencephalographic tracings. These electroencephalographic findings are consistent with transient dysfunction in the neural structures supplied by the basilar artery and its branches. Pediatric patients in whom there is a clinical picture of basilar artery migraine and who have a family history of migraine and transient electroencephalographic changes of the type herein described need not undergo invasive neurodiagnostic study.

Basilar Artery↗
PubMed1975

Seizure activity and anticonvulsant drug concentration.

Current methods permit frequent, accurate serum anticonvulsant drug concentration measurements and continuous, 24-hour electroencephalographic recording with minimal environmental restriction. These techniques were used to perform longitudinal, 24-hour recordings of electroencephalographic paroxysmal activity and sleep-wake state concurrently with frequent measurements of serum anticonvulsant drug concentrations in two patients with poorly controlled convulsions. Drug administration was designed with the intent of producing high serum concentrations at times of maximum electroencephalographic paroxysmal activity. The suppression of clinical seizures coincided with decreased numbers of paroxysmal bursts in the electroencephalogram and increased serum anticonvulsant drug concentration.

Anticonvulsants↗
PubMed1975

Dylexia in children and young adults: three independent neuropsychological syndromes.

In an attempt to delineate causal factors in dyslexia, 113 children and young adults (age-range eight to 18 years) were divided into three groups: those with brain damage who could read (n=31), those with brain damage who were dyslexic (n=53), and those without brain damage who were dyslexic (n=29). A battery of neuropsychological tests was presented to each participant. No significant differences were found between the two dyslexic groups. Three syndromes--language disorder, articulation and graphomotor dysco-ordination, and visuo-perceptual disorder--were found among the great majority of those with dyslexia. The results support a model of dyslexia as being caused by multiple independent defects in higher cortical functioning, as opposed to the theory of a single causal defect. A clinical description of each syndrome is given and models of dyslexia are discussed. The authors stress the desirability of including brain-damaged readers as a control group in any future study on causal factors in dyslexia.

Adolescent↗
PubMed1975

Basilar artery migraine in young children.

Eight children with recurrent attacks of neurologic dysfunction referable to the brainstem and cerebellum are reported. The episodes occur suddenly, clear completely, and leave the patient without residua. The most frequent signs are ataxia, alternating hemipareses, and vertigo. The majority of patients are girls, and most have the onset of the condition prior to the age of 4 years. Headache was definitely present in three children, and possibly present in four. A striking history of migraine was found in seven families, accounting for 16 affected relatives. Fifteen of these were female and 14 were on the maternal side. Follow-up of the children has not provided any evidence for progressive neurologic disease. The patient followed for the longest period of time has developed classic migraine.

Adolescent↗