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Biomedical subjects

G Neuhäuser

Publications and source records attributed to G Neuhäuser.

At least 19 recordsLinked to original sources
PubMed1989

Heterogeneity of X-linked recessive (spino)cerebellar ataxia with or without spastic diplegia.

Ataxia with spastic diplegia was seen in seven males of a Turkish family, obviously transmitted as an X-linked recessive trait. The first clinical sign in infancy was nystagmus; ataxia and pyramidal signs were noted at age 2-3 years. Patients were never able to walk. Dysarthria, orthopedic impairment, and mild mental retardation appeared later as the disorder progressed. Death occurred in the 3rd or 4th decade from infectious diseases. The syndrome resembles X-linked spinocerebellar ataxia and X-linked spastic paraplegia in some aspects but is different if compared with previously published reports. Laboratory and neurophysiological studies showed no abnormalities. Various aspects of X-linked ataxia are discussed: genetic heterogeneity is apparent from observations reported.

Cerebral Palsy↗
PubMed1986

Congenital intracranial neoplasms.

The clinical and histological findings are described in 12 infants (5 boys, 7 girls) suffering from congenital intracranial tumors. An enlarged head or signs of increased intracranial pressure were present at birth or within the first 12 months of life (average 5.1 months). The histological picture of the tumors (6 supratentorial, 6 infratentorial) varied. In 6 children a ventriculoperitoneal shunt was necessary; partial or total removal of the tumor was attempted in 5 infants; 4 patients received irradiation. Prognosis was poor, and survival time averaged 13 months. A short review of the literature is given; new diagnostic techniques (computed tomography, magnetic resonance imaging) allow earlier diagnosis. However, because of the size and location, successful treatment is rarely possible.

Brain Neoplasms↗
PubMed1979

[Sphingolipidoses].

Explore the source record for details and available documents.

Cytoplasmic Granules↗
PubMed1979

[ADAM complex--maxillofacial abnormalities and abnormalities of the extremities caused by amniotic strangulations].

The anomalies of the ADAM complex arise through amniotic strangulations, adhesions and amputations. In the face cleft formations, displacements and deformities of various structures occur. The limbs exhibit constriction grooves, secondary syndactyle or amptuations. According to observations on 8 patients and information from the literature, the presentation of Adam complexes can vary to an extraordinary degree. The anomalies result from exogenous influences. In genetic counselling phenotypically similar congenital malformations must be separated.

Abnormalities, Multiple↗
PubMed1978

Cranial computerized tomography in children with lymphoid malignancy and seizures.

Focal and generalized seizures occurred in 4 patients with acute lymphoblastic leukemia and non-Hodgkin-lymphoma. The etiology of the neurological complications could be established by cranial computerized tomography (CT): i.e., 1. localized metastasis with calcification and 2. acute intracerebral hemorrhage during induction therapy in two patients with malignant lymphomas; 3. diffuse cerebral infiltration with blast cells and 4. cerebral atrophy in two children with acute lymphoblastic leukemia who were in relapse. Accurate diagnosis of cerebral complications in hemoblastoses is essential for appropriate therapy and CT may lead to more effective treatment in patients with lymphoid malignancy and seizures.

Adolescent↗
PubMed1978

[Spongious cerebral dystrophy at an infant age (Canavan-Bogaert-Bertrand types) in three siblings of a non-Jewish family in upper Franconia (author's transl)].

A daughter and two sons of possibly consangineous parents died after motor and mental deterioration at 18, 16 and 15 months of age. Spongy degeneration of the CNS (Canavan-van-Bogaert-Bertrand type) was diagnosed on neuropathological examinationtion; the histological findings were almost identical in the patients. Own clinical experiences are compared with reports from the literature; data important in clinical and differential diagnosis are reviewed. Pathogenetical and etiological aspects are discussed; autosomal recessive inheritance has to be considered in genetic counselling.

Autopsy↗