Adult polyglucosan body disease (APBD).

Three patients aged 63, 63 and 74 years had various combinations of progressive lower and upper motor neuron dysfunction, sensory loss, urinary incontinence and dementia. Postmortem examinations in two cases showed moderate cerebral and spinal atrophy, ill-defined areas of incomplete myelin loss in white matter and small necrotic foci in the white matter of gyri, around the basal ganglia and near the dentate nuclei. The main microscopic abnormality was a massive accumulation of PAS-positive polyglucosan bodies (PB) of various sizes and shapes in the cerebral hemispheres, brainstem, cerebellum, spinal cord, nerve roots and nerves. These PB were found in the processes of nerve cells and astrocytes, but not in their perikarya. Similar PB were present in peripheral nerves and in the lungs, heart, liver and kidneys. In the third case, a nerve biopsy revealed several, unusually large, PB in the axons of myelinated fibers. These clinicopathologic features are consistent with adult polyglucosan body disease (APBD) and are distinctive from other conditions in which PB may accumulate. Twelve similar cases have been reported previously. The diagnosis can be made by nerve biopsy. The pathogenesis of APBD is not known, but it may be a polysaccharide storage disease.

Electrocorticography and stimulation.

Although acute electrocorticography (ECoG) is routinely used during epilepsy surgery there is little agreement as to its value nor criteria for its interpretation. Specific issues are reviewed on the basis of the literature and personal studies: does failure to resect the entire irritative zone prejudice seizure control, and are residual discharges predictive of failure; does activation of the ECoG by intravenous barbiturates provide information of clinical value; does intraoperative electrical stimulation help to improve localisation or avoid postoperative deficits; is the ECoG of value for monitoring functional procedures; can the value of ECoG be increased by new interpretive approaches? It is suggested that resection of the entire area of interictal discharge is not essential for satisfactory surgical outcome, but a distinction may need to be made between those discharging regions that function as pacemakers and those in which ECoG spikes appear secondarily. There is also evidence that, apart from any consideration of determining the area resected, the topography of epileptiform discharge may be predictive of pathology and surgical outcome. It is concluded that more detailed topographic and quantitative analysis of the ECoG is required before its value in planning surgery can be determined or objective interpretive criteria established.

Neuropathologic correlates of leuko-araiosis.

We describe the pathologic findings in 17 persons with dementia, 12 of whom exhibited leuko-araiosis on computed tomographic scan. The presence of white matter pallor was confirmed on autopsy in 11 of these 12 cases, 9 with Alzheimer's disease and 2 with multi-infarct dementia. Two further patients, 1 with Alzheimer's disease and 1 with multi-infarct dementia, proved to have white matter changes on pathologic examination. White matter pallor coexisted with cerebral amyloid angiopathy in the brains of the patients with Alzheimer's disease. The presence of severe white matter pallor in patients with Alzheimer's disease correlated with early death, while the presence of cortical scars was associated with prolonged survival. Because early death in patients with Alzheimer's disease has been linked with severe pathologic and chemical changes, the presence of white matter pallor may be further evidence of a particularly severe process in patients with early onset of Alzheimer's disease.

Squamous differentiation in carcinoma in situ of the cervix uteri. A cyto-histological correlation of malignant intraepithelial lesions with invasive carcinoma.

Cervical biopsies from 97 women with malignant cells in cervical scrape smears have been studied. Forty-eight patients had invasive squamous carcinoma, and 49 had intraepithelial lesions. Of these, six had dedifferentiated carcinoma in situ, nine had the differentiated lesions generally known as ;severe dysplasia', and 29 had both; the severity of the dysplasia remained doubtful in five patients. The ;severe dysplasia' was compared with invasive carcinoma. A cellular analysis of the biopsies showed that the two categories have a number of features in common, chiefly the presence of atypical and normal mitoses, nucleoli, horn cells, and giant cells. The stratification of the epithelium in ;severe dysplasia' is invariably abnormal and the architecture closely resembles that of invasive carcinoma. The cells in the smears from ;severe dysplasia' are similar to those in invasive carcinoma. It is suggested on the basis of these observations that a ;severe dysplasia' should be interpreted as a differentiated carcinoma in situ.

Cerebral malaria in the United Kingdom.

Four fatal cases of cerebral Plasmodium falciparum malaria in English travellers returning from Africa have been seen in the last 13 years. The haemorrhages, accumulations of microglia, and destruction of cerebral white matter around small veins as a result of blockage of cortical capillaries by parasitised red blood corpuscles resemble the effect of fat embolism. Microglia in the lesions is demonstrated by special neuropathological techniques. Attention is drawn to the need for a prompt recognition of malaria since appropriate treatment can be successful.

Prion protein immunocytochemistry helps to establish the true incidence of prion diseases.

Creutzfeldt-Jakob disease (CJD) and Gerstmann-Strüssler-Scheinker disease (GSSD) are transmissible spongiform encephalopathies or prion diseases affecting man. It has been reported that prion diseases may occur without the histological hallmarks of spongiform encephalopathies: vacuolation of the cerebral grey matter, neuronal loss and astrocytosis. These cases without characteristic neuropathology may go undiagnosed and consequently the true incidence of transmissible dementias is likely to have been under-estimated. Immunocytochemistry using antibodies to prion protein gives positive staining of these cases, albeit the pattern of immunostaining differs from that seen in typical forms. Accumulation of prion protein is a molecular hallmark of prion diseases, and thus a reproducible, speedy and cost-efficient immunocytochemical screening of unusual dementias may help to establish the true incidence of prion diseases.

Beta A4 deposition in the temporal cortex of adults with Down's syndrome.

The deposition of beta A4 has been quantified in the temporal cortex of 9 adults (4 male, 5 female) with Down's syndrome (DS), mean age (+/- SD) 54.7 +/- 8.8 years (range 41-67 years) at the time of death. Immunostaining with antibodies, raised to different portions of the beta A4 protein, showed a greater number of deposits than were seen with traditional silver impregnation or amyloid stains. Antibody to beta A4(1-10) identified fewer plaques than the antibody to beta A4(12-28), the mean ratio of beta A4(1-10)/beta A4(12-28) plaques being 0.30 +/- 0.10 (mean +/- SD). Morphologically, 'diffuse' and 'neuritic' deposits could be distinguished but there was no significant difference in the beta A4(1-10)/beta A4(12-28) ratio according to plaque morphology, nor did the ratio change with age. Quantitatively, the beta A4(12-28) load in the temporal cortex of DS patients was high, occupying some 14% of the field area, and it was not related to the age of the subject over the range studied. Similarly, the total beta A4(12-28) plaque count was high and not age-related. The proportion of morphological plaque types visualised by the Glees and Marsland silver impregnation and by beta A4(12-28) immunostaining were compared. In both techniques 'diffuse' plaques (D) were predominant in the younger subjects and the proportion of 'neuritic' plaques (N) increased with age. The relative proportions of cored plaques (Cp) and plaque cores (C) did not change significantly with age.(ABSTRACT TRUNCATED AT 250 WORDS)

Control of temporal lobe epilepsy following en bloc resection of low-grade tumors.

Thirty-one patients with a mean age of 18.9 years (range 3 to 53 years) who underwent temporal lobe surgery for tumor-related epilepsy over a 14-year period are presented. All had suffered chronic drug-resistant temporal lobe seizures (mean age at onset 6.9 years, range 0 to 30 years; mean duration of condition 11.9 years, range 3 to 39 years). Preoperative interictal scalp electroencephalography tracings indicated unilateral localized epileptic foci in 90% of patients, and computerized tomography scans showed abnormalities within the temporal lobe in 87%. All patients underwent en bloc temporal lobectomy. No patient received adjuvant radiotherapy or chemotherapy. Review of the histological material showed dysembryoplastic neuroepithelial tumor in 27 (87%) of the specimens and microscopic evidence of incomplete removal of tumor in 22 (71%). At long-term follow-up evaluation (mean duration 5.8 years, range 1 to 14 years), 81% of patients were completely free of seizures (Engel grade I) and 10% were almost seizure free (Engel grade II) with no deaths reported in either early or late follow-up review. Only one patient in the series failed to benefit from the surgery. Four patients suffered permanent neurological deficit causing a mild disability. Psychological assessment showed no significant fall in verbal or performance intelligent quotient for the group, but a mild memory impairment was evident in 32%. Behavioral and social aspects improved in nearly all (94%) cases. Relief of seizures could not be predicted by intraoperative electrocorticography, and outcome was independent of the completeness of tumor resection. Postoperative electroencephalographic findings identified epileptiform potentials in 65% of patients, which were associated with a worse seizure-control outcome grade.

Late fetal pontine destruction.

A child with an absent pons is described who died 8 weeks after birth. At postmortem examination, the defect was consistent with a vascular event in the last trimester.

Neuroaxonal dystrophy in the neonate. A case report.

The nervous system of a female infant who was hypotonic from birth and survived for 8 days was examined. Neuropathological and electron microscopic study revealed numerous neuroaxonal swellings, so-called spheroids, in the brainstem and extensive necrosis in the cerebral hemispheres and cerebellum.

Widespread intranuclear neuronal corpuscles (Marinesco bodies) associated with a familial spinal degeneration with cranial and peripheral nerve involvement.

Widespread intranuclear, paranucleolar, acidophilic corpuscles, known as Marinesco bodies, were found in association with degeneration of the posterior columns of ting at the age of 45 years. Neurological examination of his sister (aged 51 years) inciates that she is suffering from the same diseases. The cases appear to belong to the predominantly spinal form of spino-cerebellar degenerations or Friedreich's ataxia. This report, which includes an electron microscope examination, considers the pathological significance of Marinesco bodies.

Dementia, deep white matter damage and hypertension: 'Binswanger's disease'.

The clinical and neuropathological features are reported of 7 patients with organic intellectual impairment or dementia, hypertension and ischaemic destruction predominantly of the deep white matter of the cerebral hemispheres resembling that seen in infarction. The white matter changes have been dismissed as rare n the past, usually under the name of Binswanger's disease or subcortical arteriosclerotic encephalopathy, and without much concern for hypertension. There are now indications that this sort of case may not be uncommon. It can be suspected in life on CT scans. The accuracy of assessment of the pathological substrate of organic dementia, and therefore the strategies of research and treatment, might well benefit from further clinicopathological studies.

Pathological changes in the terminal parts of Purkyne cell axons in the dentate nucleus of the cerebellum.

This paper describes abnormal argyrophilic swellings and rings in the dentate nucleus of the cerebellum in 46 cases of various human pathological conditions. The swellings represent accumulations of neurofilaments in the terminal parts of Purkyne cell axons. They may be evidence of disturbed functions of the Purkyne cell terminals due to different causes and common to many pathological conditions. These are as varied as acute ischaemic cerebellar cortical lesions, liver failure, and chronic nervous system degenerations, such as progressive supranuclear palsy and Friedreich's ataxia, and include transmissible ones like Creutzfeldt-Jakob disease.

Trisomy 5p: a second case occurring in a previously described kindred.

A second child with trisomy 5p has been born in the kindred reported by Brimble-combe et al. The cytogenetic findings were similar to those of the index case except that the derivation was paternal instead of maternal. Improved banding techniques enabled more accurate designation of the breakpoints. The clinical and necropsy findings are described. Three non-specific phenotypic malformations and one brain abnormality, possibly specific, were common to both.