Perineurioma with ossification: a case report with immunohistochemical and ultrastructural studies.

We present the case of a peripheral nerve sheath tumor arising from the sciatic nerve in the thigh of a 42-year-old woman. Histologically, the tumor was composed of fascicles of spindle cells with extremely attenuated cytoplasmic processes. Regions of the tumor were heavily mineralized and fibrotic. The tumor was epithelial membrane antigen-positive and S100-negative. Staining for Leu-7 was positive in a patchy distribution. Type IV collagen was present between cells, and CD34 was negative in the tumor cells. Ultrastructural features included elongate cellular processes surrounded by discontinuous basal lamina and collagen. The features of the tumor therefore meet criteria for perineurioma. This rare tumor is found most often in the extremities in middle-aged woman. To our knowledge, the presence of bone formation in a perineurioma has not been described previously. The differential diagnosis includes other peripheral nerve sheath tumors, low-grade fibrous tumors, and synovial (monophasic) tumors. Evidence provided by electron microscopy and immunohistochemistry supports the diagnosis and classification of this unusual nerve sheath tumor as perineurioma with ossification. These findings expand our knowledge and criteria for classifying peripheral nerve sheath tumors.

Temporal ictal electroencephalographic frequency correlates with hippocampal atrophy and sclerosis.

We studied 328 complex partial seizures (CPS) in 63 consecutive patients with temporal lobe epilepsy who underwent scalp electroencephalography/video monitoring, magnetic resonance imaging (MRI), and surgery. The initial ictal discharge (IID), defined as the first sustained electrical seizure pattern localized to the surgical site, was determined. If the IID was rhythmic waves, the median frequency was measured. To determine if IID frequency correlates with hippocampal atrophy (HA) or sclerosis (HS), hippocampal volume ratios (HVRs) were measured (n = 52) or assessed visually (n = 11) on MRI, and mesial temporal histopathology specimens (n = 22) were graded for HS. Sixteen patients (25%) had no or mild HA (HVR = 0.78-1.02), and 47 patients (75%) had moderate-to-marked unilateral (HVR = 0.33-0.76), or bilateral, HA. Theta frequency IIDs were significantly more commonly associated with moderate-to-marked HA than were delta IIDs. Theta frequency IIDs occurred in 19% of patients with mild or no HA, and 79% of patients with moderate-to-marked HA; delta IIDs occurred in 63% of patients with little to no HA, and 13% of those with moderate-to-marked HA. In addition, the median IID frequency inversely correlated with HVR and directly correlated with HS severity. In conclusion, faster frequency rhythmic IIDs during temporal lobe CPS correlate with greater degrees of ipsilateral HA on MRI, and higher grades of HS.

Multiple subpial transections for partial seizures in sensorimotor cortex.

We report six patients with complex partial seizures arising from the primary sensorimotor cortex who underwent invasive long-term ictal electroencephalogram/video monitoring and brain mapping and then multiple subpial transections. Although four patients demonstrated no abnormalities on magnetic resonance imaging, each patient showed moderate to marked gliosis in cortex biopsied from the site of ictal onset. Extensive preoperative and postoperative neuropsychological tests demonstrated no functional deficits resulting from surgery. Only one patient failed to derive significant postoperative seizure improvement, and he subsequently underwent additional subpial sectioning without further significant improvement. We propose a modification for this surgical technique and hypothesize that these patients may represent a syndrome of central cortical epilepsy.

Transsynaptic spread of varicella zoster virus through the visual system: a mechanism of viral dissemination in the central nervous system.

We report a patient with pathologic evidence of anterograde spread of varicella zoster virus (VZV) through the visual system. A 29-year-old homosexual man developed the acquired immunodeficiency syndrome (AIDS) 2 months before the onset of left herpes zoster ophthalmicus. During the next 11 months, the zoster infection progressed to involve the left eye, with resultant keratitis, iritis, retinitis, and eventual blindness. Later, the patient developed bilateral blindness, left hemiparesis, and fatal pneumonia. At autopsy, the brain revealed destruction of the visual system and adjacent structures, with sparing of the remainder of the brain. Glial cells near the areas of necrosis showed Cowdry type A intranuclear inclusions. In situ hybridization with probes to VZV nucleic acid sequences were positive in the necrotic brain and retinal areas. Hybridization with probes to cytomegalovirus, herpes simplex virus type II, human immunodeficiency virus, and Epstein-Barr virus were negative. Electron microscopy revealed characteristic herpes group nucleocapsids. This case provides insight into the mechanisms of virus dissemination and the production of encephalitis.

Human immunodeficiency virus (HIV) infection in brains with AIDS-related leukoencephalopathy.

In addition to central nervous system (CNS) opportunistic infections and neoplasms, patients with acquired immunodeficiency syndrome (AIDS) develop unexplained dementia and encephalopathy and degeneration of the white matter. We studied autopsied brains from 20 adult patients who expired from AIDS to determine the relationship of human immunodeficiency virus (HIV) infection to white matter lesions and to clinical findings. In four patients with dementia/encephalopathy and abnormalities of the white matter, there was evidence of HIV infection as shown by in situ hybridization. In contrast, the remaining 16 patients who had no evidence of white matter degeneration revealed no hybridization to the HIV probe. The cells infected with HIV included endothelial cells, perivascular macrophages/monocytes, and multinucleated giant cells and were found in or adjacent to white matter degeneration. These results demonstrate a correlation between HIV-infected cells and AIDS leukoencephalopathy and provide further evidence for HIV-related dementia/encephalopathy.