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PubMed1990

Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome.

Stiff-man syndrome is a rare disorder of the central nervous system of unknown pathogenesis. We have previously reported the presence of autoantibodies against glutamic acid decarboxylase (GAD) in a patient with stiff-man syndrome, epilepsy, and insulin-dependent diabetes mellitus. GAD is an enzyme selectively concentrated in neurons secreting the neurotransmitter gamma-aminobutyric acid (GABA) and in pancreatic beta cells. We subsequently observed autoantibodies to GABA-ergic neurons in 20 of 33 patients with stiff-man syndrome. GAD was the principal autoantigen. In the group of patients positive for autoantibodies against GABA-ergic neurons, there was a striking association with organ-specific autoimmune diseases, primarily insulin-dependent diabetes mellitus. These findings support the hypothesis that stiff-man syndrome is an autoimmune disease and suggest that GAD is the primary autoantigen involved in stiff-man syndrome and the associated insulin-dependent diabetes mellitus. Our findings also indicate that autoantibodies directed against GABA-ergic neurons are a useful marker in the diagnosis of the disease.

Autoantibodies↗
PubMed1994

Are incremental doses of amphotericin B required for the treatment of visceral leishmaniasis?

One-hundred-and-twenty visceral leishmaniasis patients, all with demonstrable splenic amastigotes after treatment with sodium stibogluconate and pentamidine, were treated with amphotericin B. The patients were allocated into two equal groups matched by age and sex. Patients in one group received amphotericin B in the traditional incremental dose regimen, i.e. 0.05, 0.10, 0.25, 0.50 and 1.0 mg/kg body weight on days 1, 2, 3, 4, and > 4, respectively. Patients in the other group received amphotericin B at a constant 1 mg/kg bodyweight per day from day 1. Each of the 120 patients received a total dose of 20 mg/kg bodyweight. By the end of treatment the incidence of infusion-related toxicities, such as rigor and fever, and of renal toxicities, such as elevated serum creatinine and low serum potassium, was the same in both groups (P > 0.05). The two treatment regimens were also equally effective; every patient was cured and none relapsed within 6 months' follow-up. It is therefore recommended that amphotericin B be given as the full optimal dose (1 mg/kg) from day 1. There seems no advantage in the incremental regimen; not only does it 'waste' 4 days before the optimal dose is reached but it is more expensive and may encourage the development of drug resistance.

Adult↗
PubMed1981

Bites and stings of uncommon arthropods. 1. Spiders.

In addition to the Hymenoptera, less common arthropods, such as spiders and reduviids, can inflict injuries of medical importance. Identification of the source of the bite or sting may be difficult but is essential to proper treatment. Part 1 of this article describes recognition and treatment of the bites of various spiders; part 2, beginning on page 107, considers bites and stings of other arthropods.

Adult↗
PubMed1999

A veteran with acute mental changes years after combat.

A 49-year-old man presented with a five-week history of worsening confusion, agitation, and bizarre behavior. According to his mother and sister, who live with him, he had inexplicably jumped out of bed one day and complained of injuring his back. The pain apparently resolved within several days. Two weeks later, again just after awakening, he had experienced a period of confusion, lasting about 15 min. The latest episode occurred three days previously and included vivid hallucinations--at various times, he seemed to believe that he was talking to his brother on the telephone, drinking a glass of water, emptying the refrigerator, jumping into a foxhole, and stomping on rattlesnakes. He was disoriented to time as well as environment.

Antipsychotic Agents↗
PubMed2003

Transient and reversible parkinsonism after acute organophosphate poisoning.

Parkinsonism is a rare complication in patients with organophosphate poisoning. To date there have been two cases of transient parkinsonism after acute and severe cholinergic crisis, both of which were successfully treated using amantadine, an anti-parkinsonism drug. We report on an 81-year-old woman who was admitted for the treatment of acute severe organophosphate poisoning. Although acute cholinergic crisis was treated successfully with large doses of atropine and 2-pyridine aldoxime methiodide (PAM), extrapyramidal manifestations were noticed on hospital day 6. The neurological symptoms worsened, and the diagnosis of parkinsonism was made by a neurologist on hospital day 9. Immediately, biperiden (5mg), an anti-parkinsonism drug, was administered intravenously, and her symptoms markedly improved. From the following day, biperiden (5 mg/day) was given intramuscularly for eight days. Subsequently, neurological symptoms did not relapse, and no drugs were required. Our patient is the third case of parkinsonism developing after an acute severe cholinergic crisis and the first case successfully treated with biperiden. Patients should be carefully observed for the presence of neurological signs in this kind of poisoning. If present, an anti-parkinsonism drug should be considered.

Aged↗
PubMed1961

Fine structure of the cilia of rotifers.

The fine structure of the coronal cilia of the rotifer Philodina citrina has been studied in detail. Specimens were fixed with OsO(4) and embedded in butyl-methyl methacrylate, Epon 812, or Vestopal and sectioned with a Porter-Blum microtome. The details of structure of the rootlets, basal bodies, basal plates, and free cilia are described. The general structure of the rotifer ciliary apparatus conforms well to that established for other species. One of the main observations is the difference in structure of the peripheral filaments in the opposing halves of a cross-section of the free cilium. Also, in longitudinal sections evidence is offered for the existence of a helical structure in the peripheral filaments.

Basal Bodies↗
PubMed1985

Suppression of muscle contraction by vanadate. Mechanical and ligand binding studies on glycerol-extracted rabbit fibers.

The suppression of tension development by orthovanadate (Vi) was studied in mechanical experiments and by measuring the binding of radioactive Vi and nucleotides to glycerol-extracted rabbit muscle fibers. During active contractions, Vi bound to the cross-bridges and suppressed tension with an apparent second-order rate constant of 1.34 X 10(3) M-1s-1. The half-saturation concentration for tension suppression was 94 microM Vi. The incubation of fibers in Vi relaxing or rigor solutions prior to initiation of active contractions had little effect on the initial rise of active tension. The addition of adenosine diphosphate (ADP) and Vi to fibers in rigor did not cause relaxation. Suppression of tension only developed during cross-bridge cycling. After slow relaxation from rigor in 1 mM Vi and low (50 microM) MgATP concentration (0 Ca2+), radioactive Vi and ADP were trapped within the fiber. This finding indicated the formation of a stable myosin X ADP X Vi complex, as has been reported in biochemical experiments with isolated myosin. Vi and ADP trapped within the fibers were released only by subsequent cross-bridge attachment. Vi and ADP were preferentially trapped under conditions of cross-bridge cycling in the presence of ATP rather than in relaxed fibers or in rigor with ADP. These results indicate that in the normal cross-bridge cycle, inorganic phosphate (Pi) is released from actomyosin before ADP. The resulting actomyosin X ADP intermediate can bind Vi and Pi. This intermediate probably supports force. Vi behaves as a close analogue of Pi in muscle fibers, as it does with isolated actomyosin.

Animals↗
PubMed1998

Status dystonicus: the syndrome and its management.

Patients with dystonic syndromes sometimes develop increasingly frequent and relentless episodes of devastating generalized dystonia which we call status dystonicus. Twelve cases of status dystonicus, of various underlying aetiologies, are presented. Possible precipitating factors were identified in only five cases: intercurrent infection (one); discontinuation of lithium (one) and tetrabenazine (one); and the introduction of clonazepam (two). Nine patients required mechanical ventilation and three others were sedated with intravenous chlormethiazole. Drug therapy used included benzhexol, tetrabenazine, pimozide, baclofen, chlorpromazine, haloperidol, carbamazepine and acetozolamide. Two patients underwent thalamotomies, one of whom improved. Two patients died, five returned to their pre-status dystonicus condition, two eventually made a full recovery and three were worse. Patients with status dystonicus should be managed on an intensive care unit as they may develop bulbar and respiratory complications which may require ventilation. Metabolic problems encountered can include rhabdomyolysis with acute renal failure. Drug therapy with benzhexol, tetrabenazine and pimozide or haloperidol may be beneficial in some cases.

Adolescent↗
PubMed1998

Spectroscopic imaging of frontal neuronal dysfunction in hyperekplexia.

We used proton magnetic resonance spectroscopic imaging (MRSI) to assess in vivo cortical neuronal involvement in hyperekplexia. Cerebral neuronal function was measured using proton MRSI in four unrelated patients with hyperekplexia and 20 healthy controls. All patients had the major form of hyperekplexia, with additional atypical clinical features in two of them. Family history was positive in three patients and absent in one. The neuronal marker N-acetylaspartate (NAA), choline-containing compounds (Cho) and creatine (Cr) were measured in frontal, central and parietal areas. The MRSI showed a reduction of the relative resonance intensity of NAA/(Cr + Cho) in frontal and central regions in three patients, and in the right frontal region of the fourth. In one patient a second MRSI showed normal relative NAA resonance intensities over both temporal lobes as well as in the brainstem. In two subjects the topography of EEG abnormalities in the frontal lobes coincided with the MRSI findings. This proton MRSI study indicates the presence of frontal neuronal dysfunction in hyperekplexia. Whether this represents cortical dysfunction or an epiphenomenon of diencephalic or brainstem abnormalities remains open. However, the observation of normal proton MRSI in the temporal regions and brainstem in one of the patients seems to concur with the hypothesis of a facilitatory role of cortical dysfunction within areas of sensorimotor representation in the generation of the pathological startle reaction in hyperekplexia.

Adolescent↗
PubMed1975

Vestibular dysfunction in hereditary ataxia.

A kindred with a unique autosomal dominantly inherited ataxia of late onset is described. Manifestations of the condition include defective optokinetic nystagmus and absent or abnormal oculo-vestibular responses, in association with normal cochlear function. It is possible that these latter features may serve as "markers" to indicate the presence of the disease in presymptomatic young adults, thereby facilitating important genetic counseling.

Adolescent↗
PubMed1994

Evidence for recessive as well as dominant forms of startle disease (hyperekplexia) caused by mutations in the alpha 1 subunit of the inhibitory glycine receptor.

Startle disease, or hyperekplexia, is characterized by an exaggerated startle reflex and neonatal hypertonia. An autosomal dominant form of the disorder is associated with mutations in the same codon of the alpha 1 subunit of the inhibitory glycine receptor (GLRA 1) resulting in the substitution of an uncharged amino acid for Arg271 in the mature protein. However, recessive transmission is seen in the mouse mutant spasmodic which resembles startle disease phenotypically and is also associated with mutations in Glra 1. We have confirmed the finding of Arg271 mutations in individuals with startle disease in a UK family showing autosomal dominant transmission. In addition we describe an apparently sporadic case, the offspring of a consanguineous mating, who is homozygous for a novel mutation (T1112A) in GLRA 1, which results in the substitution of asparagine for isoleucine at position 244 of the mature protein. This suggests that human startle disease can display recessive as well as dominant inheritance resulting from different mutations in GLRA 1.

Adult↗
PubMed1996

Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases.

We have generated mouse models of human Tay-Sachs and Sandhoff diseases by targeted disruption of the Hexa (alpha subunit) or Hexb (beta subunit) genes, respectively, encoding lysosomal beta-hexosaminidase A (structure, alpha) and B (structure, beta beta). Both mutant mice accumulate GM2 ganglioside in brain, much more so in Hexb -/- mice, and the latter also accumulate glycolipid GA2. Hexa -/- mice suffer no obvious behavioral or neurological deficit, while Hexb -/- mice develop a fatal neurodegenerative disease, with spasticity, muscle weakness, rigidity, tremor and ataxia. The Hexb -/- but not the Hexa -/- mice have massive depletion of spinal cord axons as an apparent consequence of neuronal storage of GM2. We propose that Hexa -/- mice escape disease through partial catabolism of accumulated GM2 via GA2 (asialo-GM2) through the combined action of sialidase and beta-hexosaminidase B.

Animals↗
PubMed1984

Binding of F-actin to a region between SH1 and SH2 groups of myosin subfragment-1 which may determine the high affinity of acto-subfragment-1 complex at rigor.

Covalent cross-linking reaction between SH1 and SH2 groups in myosin subfragment-1 (S-1) by N,N'-p-phenylenedimaleimide (pPDM) was followed by the degree of inactivation of NH4+-EDTA ATPase activity. The rate of the cross-linking reaction decreased to less than a 20th in the presence of F-actin. The inhibitory effect of F-actin was not observed in the presence of MgATP. Binding of F-actin to S-1 was measured using ultracentrifugation. S-1 whose SH1 and SH2 were covalently cross-linked by pPDM or 5,5'-dithiobis(2-nitrobenzoic acid) (DTNB) did not bind F-actin. After the DTNB-cross-linked S-1 is reduced by dithiothreitol, the ability to bind F-actin is recovered. These results suggest that S-1 has a binding site for F-actin in the region between SH1 and SH2. This site appears to determine the high affinity of acto-S-1 complex at the rigor while decreasing the affinity more than 10(2) times in the presence of MgATP.

Actins↗
PubMedDate not supplied

Inheritance of porcine stress syndrome.

A total of 66 litters were farrowed in a Yorkshire herd of pigs selected for porcine stress syndrome (PSS) susceptibility. These litters included all possible combinations of matings between stress-susceptible, stress-carrier, and stress-resistant animals. When the data were analyzed by within-litter chi-square analysis, the null hypothesis of recessive inheritance could not be rejected (P less than 0.05). In addition, when the data were analyzed across litters, the null hypothesis of autosomal recessive inheritance could not be rejected (P less than 0.05).

Animals↗