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Biomedical subjects

Z Shorer

Publications and source records attributed to Z Shorer.

24 records · Page 2Linked to original sources
PubMed1993

[Rett syndrome].

Rett syndrome was described relatively recently. It affects only females and is characterized by progressive deterioration of neurological status and impairment of psychomotor development. Psychomotor development is normal in the first year of life, but then there is deterioration of fine motor and language skills and later still, of gross motor skills. This results in severe motor function handicap and dementia. The arrested head growth, severe psychomotor retardation and typical "washing hands" movements are typical of Rett syndrome. We describe a 3-year-old Bedouin girl with the clinical picture of the syndrome and review the world literature, stressing the difficulty in detection and diagnosis.

Child, Preschool↗
PubMed1987

Involvement of the peripheral nervous system in temporal arteritis-polymyalgia rheumatica. Report of 3 cases and review of the literature.

Involvement of the peripheral nervous system is very uncommon in the temporal arteritis-polymyalgia rheumatica syndrome. Three different presentations of the involvement can be recognized: mononeuropathy, polyneuropathy and brachial neuropathy--C-5 radiculopathy. We report 3 patients in whom peripheral nerve symptomatology dominated the clinical picture of the disease, and review 20 previously published cases.

Aged↗
PubMed1984

Myelinated nerve fibres and the fate of lanthanum tracer: an in vivo study.

The permeability of the marginal tight junctional system of myelin was tested in the rat employing the electron-dense tracer lanthanum nitrate. Lanthanum was either included in the fixative used for vascular perfusion (at a concentration of 20 mM) or was microinjected in vivo into the sural or tibial nerve (5, 10 and 20 mM). After 5-60 minutes, the microinjected nerves were fixed either by immersion or vascular perfusion. Lanthanum tracer was present in the intraperiod line gap of myelin, irrespective of the mode of application of the tracer, the method of fixation or the time of exposure to lanthanum. However, the tracer was present more extensively when included in the fixative compared with in vivo microinjection. Internodally, lanthanum was usually restricted to the inner, or more commonly, the outer lamellae of larger fibres, while all lamellae were usually penetrated by tracer in smaller fibres. Paranodally, compact myelin was more extensively penetrated. The periaxonal space (between axon and Schwann cell) was readily accessible to tracer. It is concluded that the marginal tight junctional system of myelin is apparently of the 'leaky' type and is permeable to ions. The findings have implications for the electrophysiology and pathophysiology of the myelinated nerve fibre.

Animals↗
PubMedDate not supplied

A new variant of autosomal recessive exfoliative ichthyosis.

We report unusual congenital ichthyosiform dermatosis in 5 of 12 children in two related families of unaffected, consanguineous Bedouin parents. It appeared shortly after birth as a fine peeling of nonerythematous skin on palms and soles. Gradually it evolved into prominent, well-demarcated areas of peeling skin in moist and traumatized regions. The cutaneous manifestations share features of ichthyosis bullosa of Siemens (IBS) and peeling skin syndrome (PSS). Histologic examination revealed orthokeratosis, a thickened granular cell layer, and spongiosis without epidermolytic hyperkeratosis. On electron microscopy there was prominent intercellular edema and numerous aggregates of keratin filaments in basal keratinocytes. This combination of clinical, histologic, and ultrastructural features has not been previously reported in the heterogeneous group of congenital ichthyoses. We suggest that it represents a new variant of exfoliative ichthyosis.

Adolescent↗
PubMedDate not supplied

[Malignant spinal hemangiopericytoma, with benign radiological appearance].

A spinal intra-dural and extra-medullary tumour is described, presenting both clinically and radiologically as a benign process. However, the pathological examination, helped by electron microscopy, concluded in favour of a malignant hemangiopericytoma. The clinical cause, after surgery and radiotherapy, confirmed this latter diagnosis.

Adult↗