Aicardi syndrome is an X-linked dominant disorder characterized by the pathognonomic association of three clinical signs: agenesis of the corpus callosum, infantile spasms, and chorioretinal lacunae. This is a severe syndrome with an estimated survival rate of 76% at 6 years and 40% at 14 years and a maximum developmental level like a 12-month-old baby in 91% of cases. We illustrate a case of Aicardi syndrome with bilateral, small chorioretinal lacunae, infantile spasms with a typical hypsarrhythmia, defects of thoracic vertebra and bilateral absence of the 12th rib associated with an arachnoid cyst of the quadrigeminal cistern, and presence of the corpus callosum with a favorable outcome.
View Article and Find Full Text PDFBackground And Purpose: The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease.
Methods: The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey.
Background: We report a rare case of arachnoid cyst incorporating choroid plexus. This 7-month-old girl presented with macrocrania. Magnetic resonance (MR) imaging disclosed a cystic lesion arising from the left prepontine cistern extending to the left middle cranial fossa.
View Article and Find Full Text PDFObject: A pressure-driven continuous jet of water has been reported to be a feasible tool for neuroendoscopic dissection owing to its superiority at selective tissue dissection in the absence of thermal effects. With respect to a safe, accurate dissection, however, continuous water flow may not be suitable for intraventricular use. The authors performed experiments aimed at solving problems associated with continuous flow by using a pulsed holmium:yttrium-aluminum-garnet (Ho:YAG) laser-induced liquid jet (LILJ).
View Article and Find Full Text PDFObject: Shock waves have not previously been used as a treatment modality for lesions in the brain and skull because of the lack of a suitable shock wave source and concerns about safety. Therefore, the authors have performed experiments aimed at developing both a new, compact shock wave generator with a holmium:yttrium-aluminum-garnet (Ho:YAG) laser and a safe method for exposing the surface of the brain to these shock waves.
Methods: Twenty male Sprague-Dawley rats were used in this study.
Case Report: A 2-year-old girl presented with repaired cloacal exstrophy and a subcutaneous mass at the midline of the lumbosacral region. Neurological examination revealed mild paraparesis with bilateral lower-extremity hyperreflexia and sensory disturbance below the level of L4. A CT scan showed asymmetrical spina bifida on the right side extending from L2 to L5, and symmetrical spina bifida below S1.
View Article and Find Full Text PDFThe authors present a case of congenital dermal sinus tract with epidermoid tumor. This 1-year-old boy was referred to the pediatric service of another hospital with recurrent pyrexia of unknown origin in April, 1999. The pediatrician found two dimples, pigmentation, and coarse hairs on the midline in his sacral region.
View Article and Find Full Text PDFIrradiation for intracranial germinoma may be associated with significant neuroendocrinological sequelae, so the establishment of lower effective doses of radiation is desirable. Eight consecutive patients with intracranial germinoma underwent combination chemotherapy with BEP (bleomycin, etoposide, and cisplatin) or PE (cisplatin and etoposide) without irradiation between 1996 and 1997. The diagnosis was based on endoscopic or stereotactic biopsy, or transsphenoidal surgery in 7 cases.
View Article and Find Full Text PDFBackground And Objectives: Making surgical incisions in vessel-rich organs without causing bleeding is difficult. Thus, it is necessary to develop new devices for this purpose, especially for surgery involving small vessels as in neurosurgery, where damage against even small cerebral vessels result in severe neurological deficits.
Study Design/materials And Methods: A laser-induced liquid jet was generated by irradiating pulsed Holmium Yttrium-Aluminum-Garnet (Ho: YAG) laser (beams of 350 microseconds pulse width) within a copper tube (internal diameter, 1 mm) with pure water (150 ml /hour).
Object: Craniopharyngiomas frequently grow from remnants of the Rathke pouch, which is located on the cisternal surface of the hypothalamic region. These lesions can also extend elsewhere in the infundibulohypophyseal axis. The aim of this study was to establish the usefulness of the frontobasal approach made through a relatively small craniotomy window for the removal of tumors protruding from the sellar-suprasellar region into the third and basal cistern.
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