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Biomedical subjects

A Koto

Publications and source records attributed to A Koto.

At least 55 records · Page 3Linked to original sources
PubMed1989

[Adrenoleukodystrophy with high signal intensity areas in bilateral pyramidal tracts from internal capsule through medullary pyramids on MRI].

We reported a case of adrenoleukodystrophy in which MRI showed high signal intensity areas in the pyramidal tracts from the internal capsule through the medullary pyramids. A 20-year-old man was admitted with complaints of slowly progressive spastic paraparesis of one and a half year duration. He had no mental deterioration, visual disturbance or sensory impairment. His maternal cousin died of adrenoleukodystrophy at the age of 13 years old, after showing progressive visual disturbance, dementia and quadriplegia. On admission, neurological examination revealed spastic tetraparesis and exaggerated deep tendon reflexes with pathological reflexes. Examination of the mental function and cranial nerves were normal. There were no sensory abnormalities in all modalities. Routine laboratory data including hematological studies, urinalysis, serum electrolytes and enzymes were all normal. Endocrinological examinations showed no adrenocortical insufficiency, and testicular function was normal. Cerebrospinal fluid, EEG, needle EMG and nerve conduction studies were also normal. CT scan showed a mild ventricular enlargement and no low density areas were seen in the cerebral white matter. Spin-echo MRI (SE 2,000/100, 2,000/40) revealed continuous high signal intensity areas in the pyramidal tracts from the internal capsule through medullary pyramids bilaterally. There were no abnormal findings in the spinal cord on MRI. Electrophysiologically, the brain-stem auditory evoked potentials (BAEPs) were abnormal and suggested the presence of bilateral dorsal brain stem lesions. Short latency somatosensory evoked potentials (SEPs) obtained by the bilateral tibial nerve stimulation revealed slowing of the central conduction time, showing delayed P37 latency and normal peripheral conduction time.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenoleukodystrophy↗
PubMed1985

Fisher's syndrome following trigeminal herpes zoster.

A case of Fisher's syndrome associated with trigeminal herpes zoster is reported for the first time. Retrograde propagation of the virus to the brainstem through the trigeminal root was thought to be the most probable pathogenic mechanism. We provide additional evidence suggesting that the focus of Fisher's syndrome is in the brainstem.

Adult↗
PubMed1984

Endothelial tubuloreticular structures in intracranial germinomas.

In studying three human intracranial germinomas tubuloreticular structures were observed within the cisterns of granular endoplasmic reticulum (RER), as well as occasionally within dilated perinuclear spaces of capillary endothelial cells. These tubuloreticular structures seen as a network of branching, convoluted, tubular profiles appear to originate from amorphous material. The development of these structures could be classified into three stages. In Stage I, the precursor substance appears as dense amorphous material within the cisterns of RER. Stage II is marked by the transformation of the amorphous material to coarse particulate material which aggregates to form tubular units. During the first and second stages, the distended RER that participates in the formation of these structures is accompanied by numerous attached ribosomes and is closely associated with mitochondria. In Stage III, the tubular units fuse with one another to form the tubuloreticular structure. In this third stage both ribosomes and mitochondria are almost absent. As a result of the almost complete disappearance of these organelles at this time, both the attached ribosomes and mitochondria may play an important role in the synthesis of the precursor substance as well as in its transformation to the tubuloreticular structure.

Aged↗
PubMed1983

Creutzfeldt-Jakob disease: a case with extensive white matter degeneration and optic atrophy.

A 52-year-old woman is described, whose clinical features were typical of Creutzfeldt-Jakob disease except for the presence of optic atrophy. Serial CT scans showed rapid development of brain atrophy early in the course. Postmortem examination revealed extensive degeneration of the cerebral and cerebellar white matter and of the optic nerves in addition to the classic findings of Creutzfeldt-Jakob disease. It is suggested that both the grey and white matter may undergo a severe destructive process early in the course of the disease, and the possibility is discussed that the white matter involvement is not a result of neuronal loss.

Brain↗
PubMed1978

Sensory neuropathy with onion-bulb formation. Report of a case with onset in infancy.

A 27-month-old girl suffered from severe sensory neuropathy with minimal motor dysfunction. The CSF protein level was increased and nerve conduction was severely impaired. Sural nerve biopsy specimen showed increased endoneurial connective tissue. An onion-bulb pattern with concentric interdigitations of Schwann cell cytoplasmic processes and redunbant basal laminae were prominent features under electron microscopy. Degress of myelination in individual fiber was far less than expected. Although the clinical manifestations of onion-bulb neuropathy with onset in infancy have been reported to resemble infantile progressive spinal muscular atrophy, the present case demonstrates that the condition can also appear as severe sensory ataxia.

Ataxia↗
PubMed1978

The Morquio syndrome: neuropathology and biochemistry.

The activity of N-acetyl galactosamine-6-sulfate sulfatase was studied for the first time in the liver and brain of a patient with a clinically typical case of Morquio syndrome with keratosulfaturia. As has been demonstrated in the fibroblasts of patients with this syndrome, this enzymatic activity was markedly decreased in both organs. Neuropathological examination revealed moderately swollen neurons containing PAS-positive, coarse globular inclusions in the cerebral cortex, Ammon's horn, basal ganglia, and thalamic nuclei. Ultrastructurally, the inclusions consisted of stacked, straight or loose, wavy membranes of various lengths, often associated with pale or moderately electron dense homogeneous "lipid droplets." These ultrastructural features of the inclusions were closely similar to the granulomembranous bodies of Hurler syndrome and the inclusions described in type B of the Sanfilippo syndrome. Unlike those mucopolysaccharidoses, however, no abnormalities were found in the gangliosides in the brain of the patient with Morquio syndrome.

Adolescent↗
PubMed1977

Syndrome of normal pressure hydrocephalus: possible relation to hypertensive and arteriosclerotic vasculopathy.

A patient with clinical features of idiopathic normal pressure hydrocephalus, who responded dramatically to shunting, was found a necropsy to have a severe hypertensive and arteriosclerotic vasculopathy with multiple lacunar infarcts. There was no pathological evidence of thickened leptomeninges, fibrosis of the arachnoid villi, or Alzheimer's disease. An abnormal absorption mechanism was demonstrated with cisternography and by an increase in the concentration of homovanillic acid in the cerebrospinal fluid. It is suggested that vascular changes may play an important role in the pathophysiology in some cases of normal pressure hydrocephalus.

Aged↗
PubMed1977

Choroidal epithelial cyst. Case report.

A supratentorial cyst in the leptomeninges of an 8-month-old infant was studied with the electron microscope. The lining of the cyst consisted of a single layer of peg-shaped epithelial cells rich in organelles and glycogen. Their free border had numerous microvilli but no cilia, and their basal portions rested on a basement membrane. Tight junctions and interdigitations were frequent between contiguous cells. The blood vessels were fenestrated. Since these features characterize developing choroidal epithelial cells, we felt the diagnosis of choroidal epithelial cyst was justified. Cysts lined by choroidal epithelium may continue to secrete cerebrospinal fluid after surgical extirpation when this is incomplete. Accumulation of the fluid in the partially excised cyst bed may therefore account for recurrence of symptoms.

Brain Diseases↗
PubMed1976

Congenital hemophagocytic reticulosis.

A fatal case of an apparently congenital form of hemophagocytic reticulosis is reported. The onset was manifested by hyperbilirubinemia and hepatosplenomegaly which were present at birth and persisted throughout life. Fever, anemia and pancytopenia developed at 1 month of age and became progressively worse. A splenectomy was performed at the age of 3 months, but the child died one day later with disseminated intravascular coagulation and pulmonary hemorrhage. The literature is reviewed with regard to the relationship of this case to (familial) hemophagocytic reticulosis and malignant histiocytosis (histiocytic medullary reticulosis). It is suggested that congenital hemophagocytic reticulosis, as described here, (familial) hemophagocytic reticulosis in infants, and malignant histiocytosis in adults all represent the same basic disorder with different ages of onset and clinicopathologic manifestations.

Autopsy↗