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Biomedical subjects

B O Berg

Publications and source records attributed to B O Berg.

11 recordsLinked to original sources
PubMed1992

Optic neuritis in children with poor recovery of vision.

We reviewed the records of 10 children with optic neuritis in whom recovery of vision was poor or incomplete. Our cases were otherwise similar to those described in previous studies in that they were always bilateral, often accompanied by a viral prodrome (seven of 10), and usually associated with disc oedema (seven of 10). Seven of twenty eyes had a final visual acuity of 6/60 or worse and only one patient regained 6/6 vision in either eye. In three patients the best vision in either eye was 6/60 or worse. Recovery of vision was often slow, taking up to six years. Five of 10 patients have developed multiple sclerosis (MS), and one child had acute disseminated encephalomyelitis (ADEM) with optic neuritis. Optic neuritis in children does not always carry a good prognosis for recovery of vision; however, the failure of vision recovery in a short period of time does not necessarily indicate a poor outcome. Some children with optic neuritis develop MS, which can develop even when optic neuritis follows a viral illness.

Adolescent↗
PubMed1991

Current concepts of neurocutaneous disorders.

The neurocutaneous diseases are a loosely bound group of clinical entities that were initially considered to have dysplastic and/or neoplastic changes of the nervous system and skin, though other organ systems are frequently involved. During the last several decades a variety of additional diseases have been included in this disease category despite their not having any known cutaneous abnormality. The major neurocutaneous syndromes are considered in this review.

Ataxia Telangiectasia↗
PubMed1989

Brother/sister pairs affected with early-onset, progressive muscular dystrophy: molecular studies reveal etiologic heterogeneity.

An autosomal recessive (AR) form of muscular dystrophy that clinically resembles Duchenne/Becker types exists, but its frequency is unknown. We have studied three unrelated affected brother/sister pairs and their families for deletions and polymorphisms with the entire dystrophin cDNA and other DNA probes from the Xp21 region to test for involvement of the DMD locus. In family 1 a large intragenic deletion was found in the affected male. The affected sister was heterozygous for this deletion, but the mother was not, implying germinal mosaicism. In family 2, no deletion was detected in the affected male. RFLP analysis revealed that the affected male and an unaffected sister shared a complete Xp21 haplotype while the affected sister had inherited a recombinant Xp21 region resulting from a crossover between pERT 87-15 and J-Bir. Only the 5' region of the dystrophin gene was shared with the affected boy. X-inactivation studies using a polymorphism in the 5'-flanking region of the HPRT gene, in conjunction with methylation-sensitive enzymes, revealed random X inactivation in the affected girl's leukocytes. In a muscle biopsy from the affected male, the dystrophin protein was present in normal amount and size. Family 3 was informative for four RFLPs detected with dystrophin cDNA probes which span the entire gene. The affected male was found to share the complete dystrophin RFLP haplotype with his unaffected brother, while his affected sister had inherited the other maternal haplotype. It is concluded that the clinical presentation of early-onset, progressive muscular dystrophy in a male and in his karyotypically normal sister can be caused by mutations at different loci. While in family 1 a deletion in the dystrophin gene is responsible, this gene does not appear to be involved in families 2 and 3.

Child↗
PubMed1985

beta-Adrenergic treatment of hyperkalemic periodic paralysis.

In a patient with hyperkalemic periodic paralysis, metaproterenol prevented muscular weakness and hyperkalemia in periods of rest after exercise. During a severe attack, the drug rapidly corrected hyperkalemia and seemed to enhance the return of strength. The action of metaproterenol may involve a beta-adrenergic-mediated increase of potassium transport via the sodium-potassium pump.

Adolescent↗
PubMed1985

Unusual neurocutaneous syndromes.

During the last several decades, the number of these rare heterogeneous disorders described as neurocutaneous syndromes has significantly increased. The criteria for inclusion have become more general, although most of the disorders have dysplastic features, many do not have a tendency for tumor formation. Further, not all of these disorders are heritable and some are reported in only one or two families. Nonetheless, there is some merit for the clinician, geneticist, and embryologist to consider these anomalies of embryologic development on some common ground. Knowledge of these syndromes is important to correctly establish the diagnosis and prognosis. In cases of the heritable disorders, genetic counseling is essential. Finally, these disorders are of theoretic importance to all biologists, as the exchange of information will be essential to ultimately unravel the reasons for their cause.

Arteriovenous Malformations↗
PubMed1979

Progressive rubella panencephalitis: immunovirological studies and results of isoprinosine therapy.

Two patients with progressive rubella panencephalitis, one with and one without stigmata of congenital rubella, were treated for 9 months with isoprinosine and showed continued clinical deterioration. Immunoviorological studies performed before, during and after treatment were unaffected by drug therapy. The virus was recovered on one occasion from the lymphocytes of one of these cases. Neither patient showed any major defects in cellular or humoral immunity. However, the lymphocytes of the patient with stigmata of congenital rubella failed to respond to rubella virus in vitro and had a heat stable, non-dialysable serum inhibitor of in vitro protein A stimulated proliferative responses. Both patients' serum interfered with the production of interferon by normal donor lymphocytes following stimulation with rubella and varicella virus antigen. Increasing serum titres of interferon which did not appear to be lymphoid or immune-specific in origin were found in these two cases.

Adolescent↗
PubMed1976

Progressive rubella panencephalitis.

A patient with progressive rubella panencephalitis developed initial symptoms of neurologic deterioration 12 years after childhood German measles. Progressive rubella panencephalitis should be considered in adolescents with progressive dementia attended by pyradmidal and cerebellar dysfunction.

Adult↗
PubMed1975

Cerebellular calcification in tuberous sclerosis.

A rarely observed pattern of cerebellar calcification was noted in the skull roentgenograms of a child with tuberous sclerosis in the absence of cerebellar dysfunction. The computerized tomographic brain scan was useful in defining the extent of the cerebellar lesion and in detecting two additional foci of calcification that were not detected by standard roentgenographic methods.

Brain↗
PubMedDate not supplied

Use of botulinum toxin to treat blepharospasm in a 16-year-old with a dystonic syndrome.

A 16-year-old boy with generalized dystonia had continuous, severe blepharospasm and facial grimacing. Local intradermal injections of botulinum A toxin greatly reduced the spasms and improved function. No side effects were observed. Local botulinum A toxin injections may be useful in the treatment of eyelid and facial spasms in patients with generalized dystonias.

Adolescent↗