PubMed · 14490110
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E R RAMEY. 1962. The reticular activating system.. https://pubmed.ncbi.nlm.nih.gov/14490110/
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We present a case of prepontine-suprasellar tuberculoma involving the premamillary region of the third ventricular floor, a site suitable to perform an endoscopic third ventriculostomy (ETV) stoma, managed endoscopically, and discuss our findings. A 5-year-old male child was admitted in an unconscious state with a history of gradually progressive symptoms of raised intracranial pressure and low-grade fever for the last 3 months. Head computed tomography showed thick enhancing basal exudates, a prepontine-suprasellar ring-enhancing lesion with consequent obstructive hydrocephalus. The child was subjected to urgent endoscopy which revealed multiple ependymal tubercles along with prepontine-suprasellar tuberculoma involving the premamillary region of the third ventricular floor. The tuberculoma was decompressed using a 5-french catheter, and ETV was performed. Postoperatively, the child improved clinicoradiologically on antitubercular chemotherapy and needed no further cerebrospinal fluid diversion surgery; he is under regular follow-up. We conclude that ETV may be attempted even in the presence of thick basal exudates and/or prepontine-suprasellar tuberculoma.
Möbius syndrome is a rare disorder characterized by congenital bilateral facial nerve palsy. Abducent palsy or other cranial nerve palsy, facial malformations, limb malformations, and skeletal malformations are common features associated with this syndrome. We report a 9-month-old infant in whom congenital muscular disorder was previously suspected because of facial muscle involvement (mask-like face), respiratory and swallowing disturbances, and hypotonia since birth. After an improvement in the respiratory infection, she showed slightly exaggerated deep tendon reflexes and an improvement in muscle tone. The occurrence of combined facial nerve palsy, glossopharyngeal nerve palsy, vagus nerve palsy, and hypoglossal nerve palsy strongly suggested that she had Möbius syndrome. Finally, the absence of the roots of bilateral facial nerves on an MRI confirmed that the disorder was Möbius syndrome. We propose that a thin slice MRI should be obtained to observe the cranial nerves around the brain stem if patients show symptoms of congenital myopathy or congenital myotonic dystrophy as well as facial nerve and other cranial nerve paralyses.
OBJECTIVES: Several large outbreaks of enterovirus 71 infections have occurred in Taiwan during the past decade. Brainstem encephalitis was the most common neurologic complication. This study was designed to determine the long-term cognitive and motor outcomes of children with enterovirus 71 brainstem encephalitis. METHODS: We conducted a prospective follow-up study of children who met the case definition for enterovirus 71 brainstem encephalitis. Subjects were stratified into isolated brainstem encephalitis (stage II), autonomic nervous system dysregulation (stage IIIa), and pulmonary edema (stage IIIb). The subjects and their parents or guardians were interviewed using structured questionnaires and received comprehensive cognitive and neurologic examinations. Motor coordination, visual-motor skill, and intellectual ability were evaluated. RESULTS: Follow-up studies were conducted in 63 previously healthy children with enterovirus 71 brainstem encephalitis (49 stage II, 7 stage IIIa, and 7 stage IIIb). The mean time to follow-up was 2.8 +/- 1.0 years (range: 1.4-4.9 years). Boys outnumbered girls by 3 to 2. The mean age at diagnosis was 2.4 +/- 1.4 years (range: 0.3-7.1 years). The most common abnormal neurologic findings on admission were altered consciousness (47.6%), followed by abnormal activities of daily living (52.4%), cerebellar dysfunction (17.5%), and cranial nerve palsy (15.9%). At follow-up, 51 of 63 children had no detectable deficits. Among the remaining 12 children, 3 died during the follow-up. The remaining 9 children (14.3%) had residual deficits. Two of these with stage IIIb disease continued to have severe motor and respiratory failure. CONCLUSIONS: Residual defects were still present in a significant proportion of children with enterovirus 71 brainstem encephalitis at >2 years after their hospitalization. Children with stage II disease were most likely to have residual cerebellar defects. Those with stage IIIb disease continued to have severe respiratory and motor impairment. Long-term follow-up of this cohort is needed to determine the ultimate prognosis.