Biomedical subjects
Publications and source records attributed to R Spiegel.
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We describe an 18-month-old child with idiopathic hypereosinophilic syndrome (IHES) who presented with fever, and cervical lymphadenopathy. Chest X-ray showed marked cardiomegaly, and echocardiogram revealed large pericardial effusion. Other causes of pericarditis were excluded. Despite the initiation of steroid therapy, signs of impending cardiac tamponade developed. Pericardiocentesis yielded bloody fluid with a white blood count of 14,800/mm3, of which 23% were eosinophils. The child recovered after pericardial drainage and prolonged systemic steroid therapy. Eosinophilic pericarditis is a rare but potentially dangerous complication of IHES.
We studied a family with pattern dystrophy of the retina (PDR) in order to elucidate the clinical course of the disorder, relations between the different forms, and the mode of inheritance. Thirty-nine family members, representing three generations, underwent a thorough ophthalmological examination, with fluorescein angiography whenever a macular abnormality was suspected. Of family members over the age of 32 years, 46.7% showed signs of PDR. We classified the clinical forms of PDR in this family into four types: minimal lesion, pseudovitelliform type, butterfly-spider type, and late-stage lesion. These forms were predominant in the order cited in age groups 31-40 years, 41-50, 51-60, and > 60 years. Significant visual loss occurred only after the age of 50 years, when 8 of 14 eyes had visual acuity of less than 20/25. Inheritance was autosomal dominant. PDR presented different clinical forms in members of this family, and in successive age classes patterns of increasing severity prevailed. Thus, the different phenotypic forms apparently represent transient stages related to the age of the patient.
Intussusception in adults is a rare entity that it is generally caused by definable intraluminal pathology. We report four cases of adult intussusception caused by lymphoma of the terminal ileum (2), an inflamed appendix (1) and a mucosal polyp (1). All presented with a variety of nonspecific and chronic symptoms, including abdominal pain, nausea and vomiting, consistent with partial small bowel obstruction. Only one patient had palpable masses in the abdomen. The most useful diagnostic radiological method was computed tomography (CT), which showed "target" lesions. The presence of the characteristic "target" lesion may obviate the need for further studies, including a barium enema. As in the cases reported here, treatment involves more than simple reduction; surgical resection is usually indicated.
The progression of Alzheimer s disease (AD) is linked with the appearance of symptoms in three key domains, namely activities of daily living (ADL), behaviour and cognition. The development and decline of these symptoms gives rise to a loss in the patient s functional capacity and contributes to the social, health care and economic costs associated with the disease. Tests suggest that the onset of these symptoms, in AD and in other types of dementia (e.g. frontotemporal dementia, dementia in Parkinson s disease and vascular dementia [VaD]), can be attributed to the loss of acetylcholine and cholinergic neurons in areas of the brain that are central to learning and memory, to execution functions and to behavioural and emotional responses, such as the cerebral cortex, the hippocampus and the limbic regions. There is evidence to show that the use of cholinesterase (ChE) inhibitors, including rivastigmine, donepezil and galanthamine, to enhance the survival of cholinergic neurotransmission is beneficial in the treatment of these symptoms. For example, administering rivastigmine stabilises and improves the performance of ADL in mild to moderate stages and slows down the decline in the capacity to carry out ADL in patients with serious AD. There is an improvement in the behavioural symptoms, the appearance of new symptoms diminishes and the use of other psychotropic drugs is reduced. Cognitive deficits become stable or improve during short term treatment and the treatment also delays the cognitive decline associated with the progression of the disease. A review of the available data reveals that ChE inhibition is beneficial in the long term in the three key symptomatic domains in different stages of the disease, as well as its perhaps being useful in different dementias. Therefore, it is likely that treatment with a ChE inhibitor improves quality of life and reduces the social and economic burden of these disorders
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