Biomedical subjects
Publications and source records attributed to F Scaravilli.
Two patients with AIDS and progressive neurological syndromes had necropsies that identified JC virus infection of the cerebral or cerebellar cortex. The unusual presentation of progressive multifocal leukoencephalopathy with grey matter involvement and normal cerebral imaging is discussed.
A study was made on the development of cortical synapses in the gracile nucleus of rats using degeneration methods. A total of 46 animals, 1 adult and 45 neonates whose ages varied from 1 to 7 days, had the right somatosensory motor cortex destroyed. The survival period varied from 1 to 30 days. Identification of axonal terminals in the gracile nucleus was also achieved by tracing the cortical fibres of 1 adult rat using horseradish peroxidase-wheat germ agglutinin (HRP-WGA). Degenerating axodendritic and axosomatic terminals that originated from cortical fibres were seen in the adult animal which survived 2 days. Their origin was confirmed by the presence of HRP-WGA inside the terminals. Light or electron microscopic changes were not seen, and in particular, the gracile nucleus was not smaller than in the control adult animals which survived 30 days or in neonates which survived 8-30 days, consistent with the small component of cortifugal fibres believed to terminate in secondary sensory nuclei. In neonates that survived a shorter period, terminal degeneration was only seen in cases operated at 4 days and later, indicating that cortical axons do not synapse in the gracile nucleus until postnatal day 4. Our results provide further support for the existence of a period in which the fibres approach their target but do not penetrate it to form synaptic junctions during the growth of cortical axons toward their targets, since previous studies have demonstrated that cortical fibres persisting to adulthood decussate completely at the level of the medulla at 12-36 h after birth.(ABSTRACT TRUNCATED AT 250 WORDS)
We counted nerve cells in different subregions of the substantia nigra in three patients with neuroacanthocytosis and compared the results with those of age-matched Parkinson's disease and control patients. Two patients with neuroacanthocytosis and clinical parkinsonism in life had a reduced neuronal density in the substantia nigra, while in a third patient without parkinsonism, this number was at the lower limit of the control range. In neuroacanthocytosis with parkinsonism and in Parkinson's disease, the ventrolateral region of the substantia nigra was most severely affected, although in neuroacanthocytosis cases, nigral neuronal loss was more widespread.
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A severe acute necrotizing herpes simplex virus type 1 encephalitis, apparently mediated by macrophages or cytokines or both, was seen in a patient with acquired immunodeficiency syndrome. The necrosis had a distribution similar to that seen in immunocompetent subjects. Viral inclusions were abundant and meningeal reaction was minimal, as previously described in one anergic patient.
The case of a 6-year-old girl who presented with a glioblastoma multiforme arising from the pineal gland is reported. Most so-called gliomas of the pineal region originate from neighbouring structures; authentic malignant gliomas of the pineal itself are extremely rare. This appears to be only the second fully documented case reported in the world literature.
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We report clinical and pathological data of 419 image-guided stereotactic biopsies. In the present series the diagnostic yield, estimated by comparing the original histological diagnoses with follow-up data, was found to be approximately 94% in gliomas. As it has been suggested that malignancy of astrocytic gliomas may be considerably underestimated in the small tissue samples obtained by stereotactic biopsy, we have reviewed 160 adult, diffuse supratentorial astrocytic tumours, correlating the original Kernohan tumour grades with survival. In this grading system, although there was generally a good correlation between histological grade and survival probability (P < 0.0001), no distinction could be made between the grade 3 (anaplastic astrocytoma) and grade 4 (glioblastoma) groups. Tumour grades obtained by applying the criteria of the more recent Daumas-Duport grading system were also determined and correlated with survival. These latter tumour grades not only correlated well with survival (P < 0.0001), but also enabled us to identify a group of patients with intermediate grade malignancy (grade 3, anaplastic astrocytoma), surviving longer than those with grade 4 tumours. Image-guided stereotactic biopsy is a useful means of providing tissue samples for histological diagnosis of brain neoplasms, including gliomas. Histological grading of adult, diffuse supratentorial astrocytic gliomas diagnosed by this technique is possible and should be carried out using an appropriate grading system.
In this retrospective study, 55 astrocytic tumours were stained with monoclonal antibody PC10 which recognizes the proliferating cell nuclear antigen (PCNA) in formalin fixed, paraffin-wax embedded sections. All the cases were graded using the Daumas-Duport system and the PCNA labelling indices (LIs) were correlated with tumour grades. Mean PCNA LI was 4.1% in the grade II, 8.1% in the grade III and 26.1% in the grade IV group. Six juvenile and cerebellar (grade I) astrocytomas had a mean PCNA LI of 5.6%. In general PCNA LI increased with tumour grade, but there was considerable variation of LIs especially in grade IV tumours which often had very high PCNA LIs (up to 72.7%). PC10 may be a useful complementary technique in surgical neuropathology, but the significance of the high LIs, seen in a proportion of glioblastomas, needs to be further determined.
A 36 year old woman presented with a subacute brainstem syndrome. MRI showed a solitary, gadolinium enhancing brainstem mass, which on biopsy showed perivenous inflammation and demyelination compatible with acute disseminated encephalomyelitis.
Three siblings presented with a progressive neurological disorder beginning in the third decade of life and characterised by palatal myoclonus, nystagmus, bulbar weakness and spastic tetraparesis. There was no evidence of intellectual deterioration or seizures. CT scan showed marked brainstem atrophy in two patients and basal ganglia calcification in one. MRI scan in one showed high signal in the brainstem and periventricular region and cerebral biopsy in this patient showed myelin loss and the presence of Rosenthal fibres. A similar disease affected the siblings' mother, maternal aunt and two of the aunt's daughters, suggesting an autosomal dominant mode of transmission of what appears to be a unique genetic disorder.
The authors report one rare case of primary adenocarcinoma of the vermiform appendix. The patient, an 83 year old female, presented with a non-pathognomonic symptomatology; diagnosis was made postoperatively. Surgery consisted of appendicectomy. The patient, due to her poor general condition, died three days after surgery for cardiocirculatory complications. In the light of the very rare case published in the literature, some diagnostic, anatomic-clinical, therapeutic and prognostic aspects of this rare tumor are discussed.
We reviewed 49 patients in whom a diagnosis of Huntington's disease (HD) seemed possible on clinical grounds, but who gave no history of definitely affected relatives. In 32 with the typical clinical features of HD (progressive chorea and dementia, postural instability, abnormal initiation of saccadic eye movements), the diagnosis was confirmed in 7 patients who had had autopsies, affected relatives were found in 5 others, and HD remained probable in a further 13 who were reexamined. In the 17 with a less typical clinical picture, a diagnosis of HD appeared most likely in 2; other causes for chorea such as cerebrovascular disease, neuroacanthocytosis, recrudescence of Sydenham's chorea, and drug-induced tardive dyskinesia could be invoked in the remainder. We conclude that the likelihood of HD in a patient with the typical clinical features of this disorder but no history of affected relatives is at least 75%, which for practical purposes implies a risk to their children hardly less than in familial HD. The most plausible explanations for seemingly sporadic patients with HD are nonpaternity and mild, late-onset disease that is overlooked by other family members.
Dyskinesias associated with dopaminergic treatment in idiopathic Parkinson's disease (PD) can be indistinguishable from those arising spontaneously in other conditions involving degeneration of, or damage to, the basal ganglia. However, those due to levodopa treatment of PD disappear on cessation of therapy. We report a patient with a clinical diagnosis of PD who, on levodopa treatment, developed hemiballism and chorea that were originally thought to be drug induced. However, the dyskinesias persisted despite stopping levodopa. Postmortem analysis showed a multisystem degeneration.
The spinal cord and the thoracic and lumbar posterior root ganglia (PRGs) of 14 HIV-positive men and 7 age- and sex-matched controls were studied by routine histology, morphometric analysis of the number of nodules of Nageotte (nN) and the diameters of sensory ganglion cells, immunohistochemistry and in situ hybridization. In 7 patients (2 of whom had evidence of cytomegalovirus ganglionitis) there were increased numbers of nN and diffuse, mild infiltration with CD45R+ T lymphocytes; no B lymphocytes were observed. Macrophages were increased in number in all cases. Whenever more than one ganglion was examined from the same patient, the appearances were similar in all. There was no alteration in the distribution of ganglion cell diameters. Changes in the spinal cord included vacuolar myelopathy (5 cases), HIV myelitis (1 case), microglial nodules (3 cases) and pallor of the gracile tracts (GTP) in 7 cases, in 6 of whom it co-existed with increased numbers of nN. Seven cases had no abnormalities, except the increase in number of macrophages in PRGs. In spite of a correlation between sensory nerve cell loss and GTP our findings suggest that other mechanisms, such as 'dying back' may contribute to the pathogenesis of GTP. Moreover, sensory disturbances were found most commonly in association with nerve cell loss; however, loss of sensory ganglion cells was not necessarily associated with evidence of sensory impairment.
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OBJECTIVE: (1) To determine whether detection of HIV proviral DNA sequences in the cerebral cortex correlates with the presence of pathological changes in this region, believed to contribute to the HIV-associated cognitive/motor complex. (2) To compare the frequency with which HIV infects cortical and subcortical regions of the brain. DESIGN: In vitro studies on HIV neurotoxicity suggest that HIV may be involved in the pathogenesis of cortical damage, recently defined as diffuse poliodystrophy (DPD) in AIDS. Previous detection of HIV antigen has localized HIV more frequently to subcortical than to cortical regions. It is not known whether HIV preferentially infects subcortical tissues or if viral expression varies in these two regions. METHODS: HIV antigen and proviral DNA sequences were detected in anterior frontal lobe tissues using immunohistochemistry (IHC) and the polymerase chain reaction (PCR), respectively. DPD was assessed by staining with antibodies against astrocytes (GFAP) and microglia/macrophages (HAM 56). RESULTS: HIV proviral DNA was detected in nine out of 15 cortical samples and in 10 out of 15 white matter samples, whilst HIV p24 antigen was localized to the cortex in three out of 15 and to the white matter in seven out of 15 cases. DPD was found in 10 cases, although in five a different aetiology may have been involved. However, DPD was present in eight out of the nine cases in which HIV proviral DNA was detected in the cortex. CONCLUSIONS: Using a non-isotopic PCR method, HIV was detected in the brains of more cases than would be expected on the basis of IHC detection, and was present in the cortex as frequently as in the white matter. HIV, together with other factors, may contribute to the pathogenesis of DPD.
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