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Biomedical subjects

J H Rees

Publications and source records attributed to J H Rees.

At least 19 recordsLinked to original sources

Cerebral haemangiopericytoma treated with conservative surgery and radiotherapy.

Cerebral haemangiopericytomas are rare tumours that resemble meningiomas but behave more aggressively, with a tendency to metastasize. We report two patients with haemangiopericytoma who had limited surgical resections owing to perioperative blood loss but who had massive tumour shrinkage after a course of radical radiotherapy. We suggest a more conservative surgical approach to the management of these tumours.

Adult↗

Aminoglycoside-associated hypomagnesaemia in children with cystic fibrosis.

Hypomagnesaemia in children with cystic fibrosis (CF) is under-recognized. We report a child with CF who developed significant hypomagnesaemia following intravenous (i.v.) treatment with aminoglycosides for exacerbations of Pseudomonas aeruginosa infection. Three additional cases have also been observed. Investigations in two patients have revealed excessive renal loss of magnesium. It is postulated that renal tubular damage secondary to the cumulative effects of repeated courses of aminoglycosides resulted in hypomagnesaemia, and we suggest screening for this problem by monitoring serum magnesium regularly in all patients with CF receiving multiple courses of aminoglycosides.

Acute Disease↗

High-grade glioma mimicking acute viral encephalitis--three case reports.

The clinical features of viral encephalitis consist of headache, fever, seizures and encephalopathy. We report three patients with high-grade gliomas presenting with encephalitic illnesses. The diagnosis of brain tumour should always be borne in mind if definite evidence for a viral infection is not obtained.

Acute Disease↗

Epidemiological study of Guillain-Barré syndrome in south east England.

OBJECTIVES: To determine the incidence, treatment, and outcome of Guillain-Barré syndrome in south east England. METHODS: Patients presenting with confirmed Guillain-Barré syndrome between 1 July 1993 and 30 June 1994 were recruited via a voluntary reporting scheme coordinated by the British Neurological Surveillance Unit, hospital activity data collected from acute admitting hospitals within the South East and South West Thames Regional Health Authorities, death certificates, and a contemporary research study of Guillain-Barré syndrome and Campylobacter jejuni infection. All patients were followed up for one year to determine outcome. RESULTS: Seventy nine patients were recruited, 35 (44%) male, 44 (56%) female, including three children (two boys, one girl). The crude (95% confidence interval (95% CI)) annual incidence was 1.2 (0.9-1.4) cases/100000 population and 1.5 (1.3-1.8)/100000 when adjusted for undetected cases. Twenty (25%) patients required ventilation for an average (SD) of 42 (64) days. Thirty six (46%) patients received intravenous human immunoglobulin, five (6%) received plasma exchange, 11 (14%) both treatments, three (4%) steroids, and 25 (32%) no immunomodulatory treatment. One year later, six patients (8%) had died, all of whom were older than 60, three (4%) remained bedbound or ventilator dependent, seven (9%) were unable to walk unaided, 14 (17%) were unable to run, and 49 (62%) had made a complete or almost complete recovery. Increasing age was significantly associated with a poorer outcome at one year. CONCLUSIONS: Despite the frequent use of modern immunomodulatory treatments Guillain-Barré syndrome still carries considerable morbidity and mortality.

Adolescent↗

Two pregnant women with vomiting and fits.

Two women with hyperemesis gravidarum were first seen with a short history of confusion diplopia, unsteadiness, and fits caused by Wernicke's encephalopathy. The neurologic presentation had been precipitated by a carbohydrate load inadvertently administered without vitamin supplementation. We stress the importance of prescribing thiamine supplements to all women with prolonged vomiting during pregnancy.

Adult↗

Reactivity of serum IgG anti-GM1 ganglioside antibodies with the lipopolysaccharide fractions of Campylobacter jejuni isolates from patients with Guillain-Barre syndrome (GBS).

Campylobacter jejuni (Cj) enteritis is the most frequently recognised infection preceding Guillain-Barre syndrome (GBS) and this combination is commonly associated with anti-GM1 ganglioside (anti-GM1) antibodies. We have examined the hypothesis that the anti-GM1 antibodies represent an immune response against the Cj lipopolysaccharide (LPS). We prepared the LPS fraction from 8 isolates of Cj, 3 from GBS patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 3 from patients with Miller Fisher syndrome (MFS) and 2 from enteritis patients without neurological disease. We looked for IgG antibodies against LPS and GM1 in the serum of 10 GBS and 10 MFS patients, including the patients from whom the Cj had been isolated, and 11 normal control subjects. The highest levels of IgG binding to LPS fractions were found in the GBS patient sera and were with one of the LPS fractions extracted from the C. jejuni isolated from a GBS patient, one from a MFS patient and two Cj isolates from enteritis patients without neurological disease. The level of IgG binding to these LPS fractions was related to the level of IgG anti-GM1 antibody in the serum. Affinity-purified anti-GM1 antibodies showed the same pattern of differential binding to the LPS fractions as the serum from which they were derived. Cholera toxin bound to the same LPS fractions as GBS patients' IgG, the binding of which was blocked by the toxin indicating specific antibody reactivity with a GM1 hapten. The presence of serum anti-GM1 antibodies did not coincide with the presence of the GM1 hapten on the LPS of the infecting strain of Campylobacter indicating that anti-GM1 antibodies do not necessarily arise as part of a simple immune response against the LPS. The IgG antibodies binding to LPS were predominantly of the IgG2 isotype but patients with anti-GM1 IgG had mainly antibodies of IgG1 subclass against both LPS and GM1, implying their production by a T-cell dependent mechanism.

Adult↗

Clinical and epidemiologic features of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is defined clinically as a peripheral neuropathy causing limb weakness that progresses for up to 4 weeks before reaching a plateau. The symptoms may be caused by inflammatory demyelination, axonal degeneration, or both. GBS occurs throughout the world, with a median incidence of 1.3 cases/100,000 population (range, 0.4-4.0). Males are more commonly affected than females, and there are peaks in young adults and the elderly. There is no clear seasonal association in Western countries, although this may be because the most frequent antecedent events, respiratory and enteric infections, have opposite seasonality. The most frequently identified cause of GBS is Campylobacter jejuni infection, which has been identified in up to 41% of patients and is associated with more severe disease and prolonged disability. Summer epidemics of GBS occur among children and young adults in Northern China and are particularly likely to be associated with C. jejuni infection.

Adult↗

Spontaneous bilateral carotid and vertebral artery dissection presenting as a Collet-Sicard syndrome.

Spontaneous dissection of the internal carotid arteries usually presents with unilateral headache, neck pain, focal ipsilateral cerebral ischaemic symptoms and a Horner's syndrome. Lower cranial nerve palsies are only rarely observed. We report a case of carotid and vertebral dissections presenting as a unilateral palsy of the ninth to twelfth cranial nerves (Collet-Sicard syndrome).

Abducens Nerve↗

Acute oropharyngeal palsy is associated with antibodies to GQ1b and GT1a gangliosides.

Three patients with acute oropharyngeal palsy had high titre anti-GQ1b and anti-GT1a IgG antibodies. No patients had ophthalmoplegia or ptosis. In all patients limb ataxia or areflexia were present without notable limb weakness. These patients describe an oropharyngeal variant of Guillain-Barré syndrome in terms of anti-GQ1b antibody reactivity and show that high titre anti-GQ1b antibodies, serologically indistinguishable from those found in Miller Fisher syndrome, can occur in a clinical setting without ophthalmoplegia. The anti-GQ1b and anti-GT1a antibody assays may be helpful tests when considering the differential diagnosis of acute oropharyngeal palsy.

Adolescent↗

Glioblastoma multiforme: radiologic-pathologic correlation.

Astrocytic tumors are divided into two basic categories: circumscribed (grade I) or diffuse (grades II-IV). All diffuse astrocytomas tend to progress to grade IV astrocytoma, which is synonymous with glioblastoma multiforme (GBM). GBMs are characterized by marked neovascularity, increased mitosis, greater degree of cellularity and nuclear pleomorphism, and microscopic evidence of necrosis. Several genetic abnormalities have been associated with the development of GBM: In some cases, the abnormality is inherited (e.g., Li-Fraumeni syndrome); in others, genetic alteration appears to result from mutation into an oncogene or deterioration of the tumor-suppressor gene p53. A common, distinctive histopathologic feature of GBM is pseudopalisading. The most common imaging appearance of GBM is a large heterogeneous mass in the supratentorial white matter that exerts considerable mass effect. Less frequently, GBM can occur near the dura mater or in the corpus callosum, posterior fossa, and spinal cord. GBM typically contains central areas of necrosis, has thick irregular walls, and is surrounded by extensive, vasogenic edema, but the tumor may also have thin round walls, scant edema, or a cystic appearance with a mural nodule. GBMs most commonly metastasize from their original location by direct extension along white matter tracts; however, cerebrospinal fluid, subependymal, and hematogenous spread also can occur. Given the rapidly growing body of knowledge about GBM, the radiologist's role is more important than ever in accurate and timely diagnosis.

Aged↗

Campylobacter jejuni infection and Guillain-Barré syndrome.

BACKGROUND: Although infection with Campylobacter jejuni is recognized as a common antecedent of the Guillain-Barré syndrome, the clinical and epidemiologic features of this association are not well understood. METHODS: We performed a prospective case-control study in a cohort of patients with Guillain-Barré syndrome (96 patients) or Miller Fisher syndrome (7 patients) who were admitted to hospitals throughout England and Wales between November 1992 and April 1994. Bacteriologic and serologic techniques were used to diagnose preceding C. jejuni infection. RESULTS: There was evidence of recent C. jejuni infection in 26 percent of the patients with Guillain-Barré or Miller Fisher syndrome, as compared with 2 percent of household controls and 1 percent of age-matched hospital controls (P < 0.001). Of the 27 patients with C. jejuni infection, 19 (70 percent) reported having had a diarrheal illness within 12 weeks before the onset of the neurologic illness. No specific serotypes were associated with Guillain-Barré syndrome. C. jejuni infection was slightly more common in men (P = 0.14) and was more likely to be associated with a pure motor syndrome and a slower recovery (P = 0.03). The patients with preceding C. jejuni infection were more likely to have acute axonal neuropathy or axonal degeneration in association with acute inflammatory demyelinating polyradiculoneuropathy, and they had greater disability after one year (P = 0.02). C. jejuni infection was significantly associated with a poor outcome even after correction for other factors associated with a poor prognosis. CONCLUSIONS: Infection with C. jejuni often precedes the Guillain-Barré syndrome and is associated with axonal degeneration, slow recovery, and severe residual disability.

Campylobacter Infections↗