PubMed · 13055070
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L KIRSTEIN. 1953-03-20. [Spasticity and rigidity].. https://pubmed.ncbi.nlm.nih.gov/13055070/
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OBJECTIVE: Our objective was to establish the role of stereotactic neurosurgical techniques in the management of brain tumours in children. MATERIALS AND METHODS: A retrospective analysis was conducted of all stereotactic procedures performed in a single centre between 1996 and 2001. The success rates of achieving histologic diagnosis and a correlation between radiologic and histologic diagnosis were examined. RESULTS: There were 7 boys and 7 girls with a mean age of 9.1 years (range: 4-15). Under general anaesthetic 15 procedures were performed in 14 patients: 12 diagnostic and 3 therapeutic; 10 with CT and 5 with MRI guidance; 10 lesions were supratentorial and 4 were in the pons. A definitive histologic diagnosis was established in 10 of the 12 cases (diagnostic yield 83%). The pre-operative radiological diagnosis was accurate for tumour type in only 75% of the cases. In 3 patients cyst aspiration was attempted: post-operatively the cyst size was decreased in 2 and unchanged in 1. Seizures and acute confusion following biopsy of a thalamic tumour occurred in 1 patient. Post-operative scans were performed in 7 patients and in 2 we noted small, clinically insignificant, haemorrhages at the biopsy site. There was no mortality and morbidity was 6.6%. CONCLUSIONS: This small series confirms that stereotactic procedures in children are safe, well tolerated, with a high diagnostic yield, which could be improved with the use of intraoperative histopathological examination.
This is a multiparametric MR study of the first reported patient with adult-onset genetically confirmed vanishing white matter (VWM) disease. It shows that, despite the presence of a severe and diffuse damage of the brain WM, brain gray matter, and cervical cord tissue, the cortical adaptive capacities were relatively preserved. Interpatient variability of brain plasticity may contribute to the known phenotypic variation of patients with VWM disease.
Holoprosencephaly (HPE) is caused by the impaired cleavage of the embryonic prosencephalon, and in the severest type, alobar HPE, the normally bilateral diencephalon and basal ganglia are fused and tend to incorporate into the upper brainstem. The detailed neuropathological features of HPE remain to be elucidated, although disturbed regulation in body temperature and electrolyte balance are frequently observed. We immunohistologically examined the expression of hypothalamic hormones, neurotransmitters, calcium-binding proteins and neuropeptides in six female autopsy cases of alobar HPE. Eight age-matched controls formed the comparative basis for the immunoreactivity of these markers during the fetal period. Neurons immunoreactive for either vasopressin or orexin-A were noted in the fused diencephalon in five HPE cases, and colocalization of vasopressin and tyrosine hydroxylase occurred in HPE cases surviving more than 6 months. Tyrosine hydroxylase-immunoreactive fibers and neurons were observed in the fused diencephalon and basal ganglia in all the six cases. Parvalbumin-immunoreactive structures were identified in the fused diencephalon and basal ganglia in five cases, and the apparent red nucleus was identified by anti-parvalbumin immunostaining in two cases aged more than 1 year. Five cases demonstrated substance P-immunoreactive structures in the diencephalon, and a substantia nigra-like structure in the midbrain was visualized by immunostainings for both tyrosine hydroxylase and substance P in four cases. Only two cases aged more than 1 year had immunoreactivity for methionine-enkephalin in the basal ganglia and substantia nigra. These data suggest that the fused diencephalon and basal ganglia exhibited functional developments in alobar HPE, and the disturbed expression of the markers may be involved in hypothalamic and/or motor abnormalities in patients.