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Biomedical subjects

T Fukusako

Publications and source records attributed to T Fukusako.

27 records · Page 2Linked to original sources

[A case of progressive myoclonus epilepsy with the first and second branchial syndrome].

A 22-year-old woman with progressive myoclonus epilepsy associated with the first and second branchial syndrome is described. Clinical features included generalized convulsive seizure, myoclonus, cerebellar ataxia and intellectual deterioration with micrognathia and malformation of auricles. She was initially suspected of mitochondrial encephalomyopathy, but the analysis of muscle biopsy and mitochondrial enzyme activities was negative. Her micrognathia and malformation of auricles were diagnosed as the first and second branchial syndrome. The case of progressive myoclonus epilepsy associated with this syndrome has never been reported, and the relationship between them remains unknown.

Adult↗

[Analysis of hemiparesis with homolateral ataxia by single photon emission CT].

Case 1. A 46-year-old man suddenly developed mild gait disturbance and left hemiparesis. On examination, gross strength was slightly reduced in the left extremities. The finger-to-nose and heel-to-knee tests disclosed moderate dyssynergia and dysmetria on the left side that could not be explained by the muscular weakness. Deep tendon reflexes were more brisk in the left extremities. There was no Babinski sign. Magnetic resonance imaging showed a region of high signal intensity in the right posterior limb of internal capsule with extension into lateral thalamus. The lesion involved the cortico-ponto-cerebellar pathway and partly the dentato-rubro-thalamo-cortical pathway. No lesions were seen in the brainstem. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion termed crossed cerebellar diaschisis by Baron et al. Case 2. A 65-year-old female developed weakness of the left extremities and gait disturbance. On examination, there was a horizontal nystagmus on lateral gaze to each side. She showed dysarthria, mild left hemiparesis and slight left hypesthesia. The finger-nose and heel-knees tests revealed moderate dysmetria and dyssynergia on the left side. Deep tendon reflexes were hyperactive in the left extremities with left Babinski sign. CT showed a low density area in the right basis pontis at about middle level. Intravenous digital subtraction angiography revealed a slight stenosis of right vertebral artery, but no other abnormality. The lesion involved the cortico-ponto-cerebellar pathway. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion. The right cerebellar blood flow was normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[A case of Cogan's syndrome associated with multiple cranial neuropathy].

A 52-year-old woman was admitted to our hospital because of disturbance of right visual acuity and double vision. At 38-year-old she became deaf bilaterally and experienced many vertigo attacks. She was diagnosed as Ménière disease. At 45-year-old vertigo attacks disappeared. At 47-year-old right peripheral facial nerve palsy developed transiently with interstitial keratitis and episcleritis of the both eyes. Oral adrenocorticosteroid therapy produced an improvement of interstitial keratitis and episcleritis. On admission, ophthalmological examination revealed bilateral interstitial keratitis and episcleritis, right retrobulbar optic neuritis and she was proven to have bilateral sensorineural deafness by otologist. Neurological examination revealed right abducens nerve paresis. Laboratory examinations revealed slightly increased erythrocyte sedimentation rate. CRP was positive. Serological tests for syphilis were negative. CSF showed mildly elevated protein level. Orbital CT scans revealed the swelling of right optic nerve. Cerebral MRI showed multiple high spotty areas in left thalamus, bilateral basal ganglia and deep white matter in T2 weighted images. After treatment with adrenocorticosteroid, right optic neuritis and abducens nerve paresis improved together with bilateral interstitial keratitis and episcleritis. Multiple cranial neuropathy may develop with Cogan's syndrome.

Abducens Nerve↗

[A case of transient bilateral primary oculomotor nerve paresis associated with head injury].

A 13-year-old boy, with no past medical history, was admitted after a car accident on October 29, 1989. On admission, he was alert. Physical examination revealed a bruise on the left frontal region, a fracture of right clavicula and right hemopneumothorax. He was treated with respirator due to dyspnea. On November 1, 1989, he was removed from respirator and expressed diplopia. Neurological examination showed bilateral ptosis, mild anisocoria, normal light reflex and horizontal gaze nystagmus in lateral gaze. Both eyes were deviated outward slightly in the primary position and showed inability to converge. The external ocular movements of both eyes were mildly limited in elevation and adduction. Ataxia was observed in bilateral upper extremities. Deep reflexes were normal and no pathological reflexes were observed. Brain CT scans showed no abnormality. On November 6, 1989, his ptosis and diplopia improved. On November 8, he was completely recovered. A magnetic resonance imaging on November 10 revealed no abnormality. We suggested that transient dysfunction of midbrain associated with head injury might cause transient bilateral primary oculomotor nerve paresis, nystagmus and ataxia.

Adolescent↗

[A case of senile onset rimmed vacuole myopathy with proximally dominant involvement].

A 73-year-old woman with progressive proximal-dominant muscular atrophy and weakness was described. She had been well until 70-year-old, when she found difficulty in standing up from sitting position. At age 72 years, she could not raise her arms. Neurological examination showed muscular wasting and weakness in the proximal parts of extremities, shoulder and pelvic girdle. In the thigh, the flexors and adductors were severely affected. Muscular weakness was also observed in m. tibialis anterior. Serum CK and aldolase were normal. Electromyography showed low voltage short duration motor unit potentials with positive sharp waves and fibrillations. Rimmed vacuoles were observed in 4.8% of muscle fibers in biopsy sample obtained from right m. quadriceps femoris. No inflammatory cells, PAS-positive materials and inclusion bodies were observed in the sample. This case differs from distal myopathy with rimmed vacuoles, because the onset was very late and her muscular weakness and atrophy was proximal dominant. This case also differs from inclusion body myositis, because muscle biopsy revealed no inflammatory cells or inclusion body.

Age Factors↗

[A case of reflex epilepsy with binasal visual field defects attack induced by family computer game].

A 12-year-old male case of reflex epilepsy with visual field defects attack induced by a family computer game was reported. This patient showed binasal visual field defects attack when he was playing a family computer game. Neurological findings and ophthalmological examinations were normal. EEG showed spike and slow wave complex distributed on right occipital region. These paroxysmal waves were activated by checkerboard pattern reversal stimuli. Stimulus intensity was closely related with these activations, but not stimulus ratio was. Examinations of brain CT and brain MRI were normal. This patient complained of discomfort without visual symptoms when he suffered checkerboard pattern stimuli. A family computer game needs mental concentration, recognition, decision, alert state of consciousness and complex finger movement. Integration of these factors may have induced visual field defects attack. It is interesting that a family computer game is the inducer of reflex epilepsy with visual symptoms. The number of reflex epilepsy is thought to increase because of prevalence of family computer games.

Child↗

[Paroxysmal itching and magnetic resonance imaging of the spinal cord in multiple sclerosis].

In three women with multiple sclerosis, paroxysmal itching occurred. We were able to detect the spinal segment lesions corresponding to the dermatome of paroxysmal itching by magnetic resonance imaging (MRI) in them. Case 1. A 38-year-old woman was admitted with chief complaints of tingling sensation in the left side of the body, left hemiparesis and paroxysmal itching in the neck and left upper extremity. Examination on admission revealed left hemiparesis, mildly exaggerated deep tendon reflexes in the left upper and lower extremities, positive Lhermitte's sign. Superficial sensation was decreased and dysesthetic below the left C3 segment. Vibration and joint sense were moderately decreased in the left upper limb. Painful tonic seizure-like attack occurred in the neck bilaterally. Paroxysmal itching occurred in the neck and left upper extremity corresponding to the cervical spinal segments bilateral C3, left C4 to C6. MRI revealed multiple high signal intensities in the white matter of the cerebral hemispheres, the medullo-cervical junction and the cervical segment C3 to C4 in T2-weighted spin-echo images. The C3 to C4 lesion was found in the left dorsal area of spinal cord in axial image. High signal areas of cervical cords on T2-weighted spin-echo images were reduced in response to adrenocorticosteroid therapy, and paroxysmal itching disappeared. Case 2. A 24-year-old woman complained chiefly of mild tetraparesis and left hand clumsiness. On admission, she had right central facial palsy, mild weakness of all limbs, painful tonic seizure of left upper limb, positive Lhermitte's sign and bilateral Babinski sign. Superficial sense was mildly decreased and dysesthetic in left upper extremity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Rigid spine syndrome associated with cardiomyopathy: clinical and nosological considerations.

We report observations in a 32-year-old man with the following characteristics of rigid spine syndrome: humero-peroneal muscular atrophy and weakness; bradycardia, dilated cardiomegaly and complete cardiac conduction block; and severe fatty degeneration of the paravertebral and calf muscles. The latter showed a predominance of type 1 fibers, a deficiency of type 2A fibers, and an increase in type 2C fibers. The patient had no familial background of the disease. There was no contracture of the elbows. These findings, especially the severe cardiac involvement, suggest that the rigid spine syndrome can be difficult to distinguish from the Emery-Dreifuss form of muscular dystrophy.

Adult↗