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Biomedical subjects

J Gawler

Publications and source records attributed to J Gawler.

At least 19 recordsLinked to original sources

Heightened intrathecal release of proinflammatory cytokines in Creutzfeldt-Jakob disease.

The authors report high intrathecal release of tumor necrosis factor alpha (TNF-alpha) and interleukin (IL)-1beta in five patients with sporadic or new-variant Creutzfeldt-Jakob disease (CJD) without activation of the humoral or lymphocytic immune responses. Increased release of TNF-alpha and IL-1beta was also detected in some patients with progressive dementias. CJD is associated with a local cerebral host response that involves the release of proinflammatory cytokines.

Creutzfeldt-Jakob Syndrome↗

Neurological illness following treatment with fludarabine.

Fludarabine is a comparatively new drug for the treatment of low-grade lymphoid malignancy. This report describes five cases of unusual neurological illnesses occurring after treatment with fludarabine. These suggest that caution should be exercised in patients receiving fludarabine who develop neurological abnormalities, with prompt investigation and if necessary cessation of the drug.

Adult↗

A familial disorder associated with palatal myoclonus, other brainstem signs, tetraparesis, ataxia and Rosenthal fibre formation.

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.

Adult↗

Brachial plexopathy in breast carcinoma--an unsolved problem.

Brachial plexus involvement from carcinoma of the breast produces a severe disability which presents difficulties in diagnosis and treatment. Five patients who suffered this complication are described and the problems they present are discussed.

Adult↗

Peripheral neuropathy associated with Castleman's disease.

Four patients with polyneuropathy complicating the plasma cell variant of Castleman's disease (angiofollicular lymph node hyperplasia) are described. The neuropathy was predominantly motor and severely disabling. Vasculopathy, papilloedema, organomegaly, endocrinopathy, oedema and paraproteinaemia were variably present in these patients. Sural nerve biopsy showed changes of both demyelination and axonal loss. Capillary proliferation and endothelial hypertrophy in the epineurium and endoneurium, similar to that seen in affected lymph nodes, suggested that a diffuse vasculopathy may contribute to the neuropathy. Serum antibody activity against a variety of neural antigen preparations was not detected in any of the patients. Two untreated patients died. Substantial improvement in the neuropathy occurred in the two patients treated with cyclophosphamide and prednisolone.

Adult↗

Minimal neuropsychological sequelae following prophylactic treatment of the central nervous system in adult leukaemia and lymphoma.

The potential long-term toxicity of central nervous system prophylaxis (CNS-P) in adult acute lymphoblastic leukaemia (ALL, n = 17) and non-Hodgkin's lymphoma (NHL, n = 7) was investigated in a multidisciplinary study. At least 4 years had elapsed from CNS-P (mean 11.5 years) for all patients. Neurological history and physical examination were unremarkable; minor signs were commoner in older patients (P less than 0.02). Psychometry yielded normal results, but individual verbal IQ generally exceeded performance IQ, with a trend to more marked differences in younger adults (P = 0.06). EEG was scored and differed significantly from that of controls, with a tendency to more marked (but still minor) abnormalities in younger patients (P = 0.06). Brainstem auditory evoked potentials demonstrated significant but generally minor abnormality in 24% of patients. CT brain scan revealed widening of cerebral hemisphere sulci to greater than 3 mm in 38% of patients; cerebral atrophy was commoner in the older group (P less than 0.02) and those with neurological signs (P less than 0.02). MRI brain scans were normal in all patients tested. Thus, following standard CNS-P for ALL at this hospital, there is a 5% primary CNS relapse rate, and only minimal, mainly subclinical, long-term neuropsychological toxicity.

Adult↗

Serial cerebral CT abnormalities in relapsing acute disseminated encephalomyelitis.

A 7 year old girl developed acute disseminated encephalomyelitis following a Mycoplasma pneumoniae respiratory infection. The illness followed a relapsing course during the first two months. Computed tomography (CT) showed cerebral lesions of a severity and extent out of proportion to the clinical manifestations. The CT abnormalities altered with changes in her clinical state.

Child↗

Comparison of the measurements of the cerebral ventricles obtained by CT scanning and pneumoencephalography.

This study was undertaken to compare the accuracy of measurements of the venticular system obtained by the CT scanner and pneumoencephalography (PEG). In it the ventricular system was evaluated in each case by measuring the span of the frontal horns, cellae mediae and third ventricle in relation to the diameter of the inner and outer tables of the skull from the PEG films. The indices of Evans, Schiersmann, and Schaltenbrand and Nürnberger were applied to these measurements. The same indices were obtained using the appropriate CT printouts. A very good measure of agreement was obtained with the indices arrived at from both the CT scan printouts and the pneumoencephalograms.

Adolescent↗

Tuberous sclerosis. Comparison of computed tomography and conventional neuroradiology.

Sixty-two patients with tuberous sclerosis were studied by skull radiography. In addition, 16 had CT scanning, 26 had air studies, and 5 had cerebral angiography. Both subependymal and parenchymal tubers were shown on CT; all had a higher density than the brain and none were enhanced by contrast material. CT showed tubers in 13 of 16 patients, including 4 of 6 patients with glioma and hydrocephalus. Air studies showed subependymal tubers in 12 of 26 patients. Four other patients had intraventricular gliomas but no tubers. Skull radiographs showed spotty calcification, compatible with tuberous sclerosis, in 30 cases. Four patients had calcification compatible with tumor. Angiograms were nonspecific, showing intraventricular masses but no tubers.

Air↗

Computerized tomography (the EMI Scanner): a comparison with pneumoencephalography and ventriculography.

Computerized tomography, using the EMI Scanner, allows the diagnosis of cerebral atrophy or hydrocephalus to be made with the same degree of accuracy as conventional neuroradiological methods. Ventricular measurements made on EMI scans have been compared with those from pneumoencephalograms and ventriculograms. A range of normal ventricular measurements for the EMI scan is suggested.

Adolescent↗

Intracranial epithelial cysts. Report of two cases.

The authors report two cases with large unilocular intracerebral epithelial cysts. Diagnosis was facilitated in both patients by computerized tomography (EMI scanner). The clinical and diagnostic aspects of previously reported cases are reviewed, and the etiology and pathogenesis of these cysts discussed.

Adult↗