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Biomedical subjects

S Wakai

Publications and source records attributed to S Wakai.

At least 91 records · Page 5Linked to original sources
PubMed1996

"Obtundation status (Dravet)" caused by complex partial status epilepticus in a patient with severe myoclonic epilepsy in infancy.

PURPOSE: We report a 1-year 7-month-old boy with severe myoclonic epilepsy in infancy (SME) who exhibited complex partial status epilepticus (CPSE), which was confirmed by ictal video-EEG analysis. This boy first had a hemiconvulsion in a hot bath at age 3 months. Thereafter, he exhibited both partial and generalized seizures that were extremely intractable. At age 9 months, he had a prolonged episode of impaired consciousness that fit the "obtundation status in SME" defined by Dravet et al. METHODS AND RESULTS: Ictal EEG revealed persisting irregular spike-and-wave complexes over the left hemisphere, predominantly in the occipitotemporal area, and confirmed CPSE. The EEG abnormalities with decreased level of the consciousness continued approximately 6 h after onset of the symptoms even with AED administration. CONCLUSIONS: Because SME features both generalized and focal seizures, both types of nonconvulsive status may be seen in SME. Although Dravet et al. already reported long-lasting atypical absences in patients with SME as "obtundation status," we demonstrated CPSE in an infant with SME who exhibited a prolonged stuporous state.

Coma↗
PubMed1996

[Ruptured peripheral lenticulostriate artery aneurysm in a child: case report].

We report a 12-year-old with intracerebral hematoma and subarachnoid hemorrhage secondary to the rupture of a peripheral lenticulostriate artery aneurysm. We carried out proximal clipping of the lateral lenticulostriate artery at the middle cerebral artery following six days of continuous ventricular drainage for acute hydrocephalus. Postoperative angiography confirmed the obliteration of the aneurysm. The girl was discharged without any neurologic deficits. To our knowledge, this is a first reported case in the literature of a ruptured peripheral lenticulostriate artery aneurysm in childhood. The pathogenesis of such an aneurysm and the treatment modalities are discussed.

Aneurysm, Ruptured↗
PubMed1995

Brain tumors associated with infantile spasms.

Two patients with brain tumors associated with infantile spasms are reported. Both infants displayed typical clinical features of infantile spasms, comprising tonic spasms manifesting in series and hypsarrythmia. In Patient 1, magnetic resonance imaging revealed a tumor in the hypothalamic region, suggestive of hypothalamic hamartoma. In Patient 2, cranial computed tomography and magnetic resonance imaging indicated the existence of a primary brain tumor with calcification in the right temporal lobe. Adrenocorticotropic hormone therapy combined with clonazepam relieved seizures in both infants. In Patient 1, resection of the hypothalamic tumor is impossible because the tumor lacks a stalk. In Patient 2, pathologic investigation of removed tumor tissue demonstrated mixed-oligoastrocytoma. It is suggested that focal lesions, like those in our patients, are involved in the development of infantile spasms.

Brain Neoplasms↗
PubMed1995

Complex partial status epilepticus in childhood.

We report 5 pediatric patients (2 male, 3 female; age range: 4-8 years) with complex partial status epilepticus (CPSE). Four patients had previous illnesses and mild motor or mental retardation. In 2 patients, CPSE was induced by inappropriate management or selection of antiepileptic drugs. Clinical features varied and automatisms were observed in 3 patients. In 1 patient, decreased physical tone with syncope and impaired consciousness with amaurosis were observed. The episodes of CPSE were continuous in 3 patients and recurrent in 2 patients. In 4 patients, ictal electroencephalographic (EEG) findings, including video-EEG analyses of 3 patients, demonstrated persistent focal epileptic features. Intravenous diazepam abolished CPSE in 3 patients with brief periods of definite EEG localizations remaining. In 4 patients, seizure prognoses were favorable after appropriate treatments; in 1 patient, seizures were intractable even after antiepileptic drug administration.

Anticonvulsants↗
PubMed1995

Expanding intracerebral hematoma from pituitary adenoma: case report.

We report the case of a 53-year-old man presenting with a headache of sudden onset and blurred vision secondary to hemorrhage from a nonfunctioning pituitary adenoma, which had been treated 25 years previously by transcranial surgery and postoperative irradiation. The intratumoral and intracerebral hematoma expanded to three times its initial size because of a recurrent hemorrhage that occurred during a 2-day period while the patient awaited surgery. The mechanisms of hematoma expansion and the management strategy in such a case are discussed, and a review of the pertinent literature is presented.

Adenoma, Chromophobe↗
PubMed1995

Medullary hemorrhage.

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Arteriovenous Malformations↗
PubMed1994

[Clinicopathological features of dysembryoplastic neuroepithelial tumor (DNT): four cases report].

We reported four cases of dysembryoplastic neuroepithelial tumor (DNT) with intractable complex partial seizures, and analyzed their clinical and pathological features. The age of patients ranged from four to nineteen years old. Three were boys and one was a girl. The age of seizure onset ranged from eight months to five years. They did not have any neurological deficits. In all cases, CT scan revealed a low density mass in the temporal lobe. Two cases demonstrated calcifications, and another two cysts. The mass demonstrated low signal intensity in T-1 weighted MR images with mild contrast enhancement by Gd-DTPA. Long-term video-EEG monitoring revealed the epileptogenic focus in the temporal lobe on the side of the mass lesion. Anterior temporal lobectomy with total removal of the tumor was carried out in all cases. Hippocampectomy was performed in three cases in which intraoperative electrocorticogram demonstrated spike discharges in the hippocampus. In the surgical specimens, the tumor consisted of increased astrocytes, oligodendrocytes, and some neurons. A cortical nodule appeared in one case, and cortical dysplasia appeared in two cases. It was difficult to distinguish the tumor of case 3 from ganglioglioma because of mild neuronal atypia. The tumor of case 4 demonstrated increased capillary vessels and was also difficult to differentiate from vascular anomalies. The pathological diagnosis of DNT was still difficult. Consequently, we speculated that DNT included some transitional types between congenital anomalies and ganglioglioma.

Adolescent↗
PubMed1994

[Massive intraventricular hemorrhage from prolactinoma during bromocriptine therapy: case report].

We are reporting herein a case with massive intraventricular hemorrhage secondary to prolactinoma, which initially extended into the suprasellar cistern but reduced in size after one year of bromocriptine therapy, resulting in intrasellar herniation. This is the 6th reported case of intraventricular hemorrhage from a pituitary adenoma. Neuroradiographic features are described in detail and the pertinent literature is reviewed.

Administration, Oral↗
PubMed1994

Lobar hemorrhages.

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Cerebral Amyloid Angiopathy↗
PubMed1993

Central nervous system involvement and generalized muscular atrophy in occipital horn syndrome: Ehlers-Danlos type IX. A first Japanese case.

Occipital horn syndrome (OHS, Ehlers-Danlos syndrome type IX) belongs to the category of the copper metabolism disorders and is at present being investigated biochemically as is Menkes' disease. Unlike Menkes' disease, most patients with OHS have mild submentality. We report a case of OHS with severe central nervous system involvement and muscular atrophy in a 34-year-old male. He had psychomotor retardation and seizures since early childhood and now presented severe mental retardation and generalized muscular atrophy in addition to characteristic facial appearance, hyperelasticity of the skin and joint subluxation. Laboratory investigations revealed a low serum copper and ceruloplasmin level as well as intestinal non-absorption of copper. Radiographic imaging showed occipital exostoses, bladder diverticula, tortuosity of the peripheral vein and osteoporosis of the skeletal bones. The activity of lysyl oxidase, a copper-enzyme involved in cross-link formation in collagen, was found to be decreased in a skin-biopsy specimen. Electron-microscopic investigation of a muscle biopsy showed irregularity of the myofibrillar network and accumulation of concentric laminated bodies in the subsarcolemmal regions.

Adult↗
PubMed1993

Medulloblastoma associated with malignant rhabdoid tumor of the kidney: case report.

The authors report a case of 6-month-old boy with cerebellar medulloblastoma associated with malignant rhabdoid tumor of the kidney, presenting with an abdominal mass, large head and projectile vomiting. Following removal of the renal tumor, the mass arising from the superior vermis about 6 cm in diameter was removed by a combined right occipital transtentorial and suboccipital approach. The patient had been well for 3 months after surgery followed by chemotherapy but died eventually of tumor recurrence in the abdomen. Reported cases of malignant rhabdoid tumor of the kidney associated with brain tumor are reviewed and its characteristics are discussed.

Cerebellar Neoplasms↗