Prognostic limitations of the Daumas-Duport grading scheme in infratentorial neuroglial tumors in children.

The Daumas-Duport grading scheme (DDGS) utilizes four histologic features in an additive method (grade 1 if none present, grade 2 if only one is present, etc.). Its efficacy in achieving prognostically homogeneous groups of childhood infratentorial neuroglial tumors and its concordance with World Health Organization (WHO) diagnoses has not been evaluated.

A rare case of lymphangiomatosis of the craniocervical spine in conjunction with a Chiari I malformation.

Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected. Neurosurgical consultation may be called for several reasons: (1) lesions involving the calvarium and/or spine; (2) nondiagnostic biopsies from more accessible and less morbid locations, and (3) persistent CSF leak and/or recurrent meningitis.

Castleman's disease in a child presenting with a partly mineralized solitary meningeal mass.

We report a case of solitary intracranial childhood Castleman's disease (CD) presenting with a sudden onset of partial seizures due to a meningeal and cortical mass lesion. The patient was a previously healthy 8-year-old girl who developed a new onset of simple partial seizures with motor signs. On physical examination, she was neurologically intact.

Absence of hippocampal sclerosis in children with multiple daily seizures since infancy.

We report a series of nine children with multiple daily seizures since infancy who underwent functional hemispherectomy that included en bloc resection of the hippocampus and the temporal neocortex. In all cases, the hippocampi were normal by conventional histology despite the fact that these patients had suffered from recurrent seizures over a long period of time. This observation suggests that extremely frequent seizures in childhood are not invariably associated with the development of hippocampal sclerosis.

HIV-1 and its causal relationship to immunosuppression and nervous system disease in AIDS: a review.

Acquired immune deficiency syndrome (AIDS), caused by human immunodeficiency virus type 1 (HIV-1), has claimed more than 10 million lives over the past 15 years. There are approximately 30 million HIV-positive people worldwide, 89% of whom reside in sub-Saharan Africa and Asia. The intricate relationship between the virus and HIV-related human multisystem pathology prompted scientists to modify many previously established concepts about infectious diseases and immunology, and to test new ones.

Neocortical dendritic pathology in human partial epilepsy: a quantitative Golgi study.

We used a computerized image-analysis system to perform a quantitative analysis of rapid Golgi-impregnated pyramidal neurons of the third cortical layer of histologically normal cerebral cortex surgically removed from patients with partial epilepsy. Various parameters of 51 neurons from 9 patients and 29 neurons from 5 age-matched controls were compared. Dendritic spine density decreased progressively with increasing duration of seizures, and dendritic swellings were most numerous in epilepsy cases of uncertain etiology and in patients with seizures of longer standing.

Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington's disease: a quantitative Golgi study.

Aided by a computer microscope, the Eutectic Neuron Tracing System, we performed a quantitative analysis of 59 rapid Golgi-impregnated pyramidal neurons from the third and fifth prefrontal cortical layers (P III and P V neurons) in tissue sections obtained from seven autopsied Huntington's disease (HD) patients (grades 2 through 4) and 59 corresponding cells from eight age-matched control cases. Relative to controls, P III HD neurons had a significant increase in the number of primary dendritic segments arising from soma, total dendritic length, and total surface area. The HD cells also had significantly more dendritic branches at three intervals of measurement in a Sholl diagram (100 microns, 200 microns, and 400 microns from the soma) and a significant increase in the number of dendritic branching points.

Granulomatous amebic encephalitis in an AIDS patient.

A 39-year-old man with AIDS died after developing a variety of neurologic symptoms and signs. CT showed multiple enhancing lesions in the cerebral hemispheres and cerebellum. Postmortem examination revealed parenchymal hemorrhagic and necrotizing lesions with a thrombo-occlusive vasculitis due to Acanthamoeba, which was typed as Acanthamoeba group 2, probably A rhysodes, by immunofluorescence.

Distribution of vascular permeability factor (vascular endothelial growth factor) in tumors: concentration in tumor blood vessels.

Vascular permeability factor (VPF) is a highly conserved 34-42-kD protein secreted by many tumor cells. Among the most potent vascular permeability-enhancing factors known, VPF is also a selective vascular endothelial cell mitogen, and therefore has been called vascular endothelial cell growth factor (VEGF). Our goal was to define the cellular sites of VPF (VEGF) synthesis and accumulation in tumors in vivo.

Morphometric analysis of the prefrontal cortex in Huntington's disease.

We performed a morphometric analysis of cresyl violet-stained sections from the dorsolateral prefrontal cortex of 81 patients with Huntington's disease (HD) (grades 2, 3, and 4) and 23 age-matched normal controls. We counted large pyramidal neurons, small neurons, astrocytes, oligodendroglia, and microglia under the guidance of a specifically predefined set of morphologic criteria for each cell type and recorded the thickness of each cortical layer. Our results demonstrate a selective and progressive loss of a subset of the large pyramidal neurons in cortical layers III, V, and VI of HD patients, and a decrease in the thickness of the respective cortical laminae.

Spinal leptomeningeal metastasis from cerebral glioblastoma. Appearance on magnetic resonance imaging.

A case of circumferential leptomeningeal metastasis to the spinal cord from an intracranial glioblastoma multiforme (spinal meningeal gliomatosis) is presented. The clinical, radiographic, and pathological features are described. Spinal magnetic resonance imaging with gadolinium-diethylenetriaminepentaacetic acid accurately demonstrated the spread of disease when compared with autopsy findings.

Ultrastructure of blood vessels in the ganglionic eminence of premature rabbits with spontaneous germinal matrix hemorrhages.

Approximately 20% of preterm rabbit pups develop spontaneous germinal matrix hemorrhages (GMH). To understand better the pathogenesis of GMH we studied the ultrastructure of germinal matrix (GM) blood vessels in rabbits delivered at gestational day 28. Regardless of luminal size, the walls of most GM vessels had the structural characteristics associated with a blood-brain barrier (BBB) and consisted of endothelial cells and pericytes, surrounded by GM cell processes.

Chronic myopathy with a partial deficiency of the carnitine palmityltransferase enzyme.

To date, chronic myopathy has not been reported (to our knowledge) to occur in carnitine palmityltransferase (CPT) deficiency, a disorder of muscle lipid metabolism. We describe two patients with CPT deficiency: a mother, who had a partial CPT deficiency associated with fixed proximal weakness but without rhabdomyolysis, and her son, who had a complete CPT deficiency (95% reduction in enzyme activity) and who suffered from classic attacks of exercise-induced rhabdomyolysis but had normal strength on recovery. Careful examination of family members of patients with complete CPT deficiency is suggested in order to identify clinically affected heterozygotes.

Echogenic vasculature in the basal ganglia of neonates: a sonographic sign of vasculopathy.

The vessels that supply the basal ganglia and thalami are not normally conspicuous on the cranial sonograms of neonates. Twelve neonates with abnormally echogenic or "bright" vessels on cranial sonograms were studied. Records of these 12 patients were reviewed and were correlated with the neuropathologic findings available in four.

Intracerebral and intracerebellar neurilemoma.

Neurilemomas (schwannomas) within the brain substance and not associated with a peripheral nerve are rare. We have presented two such cases, one with a prominent angiomatous component. Neither patient had neurofibromatosis.

Adult onset Hallervorden-Spatz disease with neurofibrillary pathology. A discrete clinicopathological entity.

Three adults with progressive cognitive decline and extrapyramidal dysfunction were studied. They were all mentally retarded women without known chromosomal abnormalities, ranging in age at the time of onset from 31 to 42 yrs with an average duration of illness of 6 yrs. Neurological signs were stereotyped and consisted of a unilateral equinovarus foot posture followed by progressive dementia, rigidity and quadriparesis.

Meningeal melanocytoma. Case report and review of the clinical and histological features.

A melanin-producing, encapsulated, histologically benign-appearing tumor of the trigeminal nerve was surgically removed from a 9-year-old boy. It recurred 6 months later and was then subtotally resected and treated with radiation therapy. The clinical and pathological features of this and 10 similar cases reported in the literature are reviewed.

Progressive polyradiculopathy in acquired immune deficiency syndrome.

We studied three patients with acquired immune deficiency syndrome (AIDS) and progressive polyradiculopathy. Postmortem examination of one patient disclosed extensive necrosis, inflammatory infiltrates, and focal vasculitis of spinal roots. Typical cytomegaloviral (CMV), intranuclear, and intracytoplasmic inclusions were noted within enlarged endoneurial and endothelial cells.

Thrombotic cerebral vasculopathy associated with herpes zoster.

We describe the clinical, radiographic, and pathological findings in 3 patients with large-vessel cerebral vasculopathy following herpes zoster. Two of the patients were studied at postmortem examination, and a brain biopsy was performed in the third. Each of the 3 patients suffered thrombotic occlusions of large vessels without notable inflammatory or granulomatous changes following trigeminal or segmental herpes zoster infection.