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J J Hallett

Publications and source records attributed to J J Hallett.

9 recordsLinked to original sources

Anti-striatal antibodies in Tourette syndrome cause neuronal dysfunction.

Serologic studies of children with Tourette syndrome (TS) have detected anti-neuronal antibodies but their role in TS has not been explored. Stereotypies and episodic utterances, analogous to involuntary movements seen in TS, were induced in rats by intrastriatal microinfusion of TS sera or gamma immunoglobulins (IgG) under noninflammatory conditions, as found in TS. Immunohistochemical analysis confirmed the presence of IgG selectively bound to striatal neurons. These data support the hypothesis that binding of an anti-neuronal antibody from some children with TS induced striatal dysfunction and suggest a possible cause for the basal ganglia alterations observed in children with TS.

Adolescent↗

Antibodies against a neuron-like (HTB-10 neuroblastoma) cell in children with Tourette syndrome.

BACKGROUND: Similar to the model for Sydenham's chorea, antineuronal antibodies (ANAb), which develop in response to a preceding streptococcal infection, have been speculated to have a role in the development of Tourette syndrome (TS). METHODS: Serum antibodies against the neuron-like HTB-10 neuroblastoma cell were assayed by ELISA methods and Western blot analysis on 41 children with TS (mean age 11.3 years) and 39 control subjects (mean age 12.1 years). RESULTS: Group comparisons of ELISA assay optical density (OD) showed that mean OD values for serum antibodies were not different [control (mean +/- SEM), .506 +/- .076; and TS, .584 +/- .053 (p = .38)]. In contrast, median values [.353 in control subjects and .477 in TS subjects (p = .012)] were significantly different. Western blots identified numerous bands in all TS and control sera with no difference in identified HTB-10 antigens. There was no relationship between the presence of ANAb and age of tic onset, family history, tic severity, attention deficit hyperactivity disorder, or obsessive compulsive disorder. No relationship existed between positive strep titers (ASO > or = 166 and/or antiDNAaseB > or = 170) and ANAb determinations or the severity of tics. CONCLUSIONS: Children with TS have higher median, but not mean, levels of ANAb, as measured by the HTB-10 neuroblastoma cell membrane assay. This assay system identified antibodies in both control and clinical groups and failed to identify a relationship between antibodies and clinical phenotype or one-time markers for streptococcal infection. Further studies are required to define a possible immune-mediated hypothesis for TS.

Adolescent↗

Antibodies against human putamen in children with Tourette syndrome.

BACKGROUND: Similar to the model for Sydenham's chorea, antineuronal antibodies, which develop in response to a preceding streptococcal infection, have been speculated to have a role in the development of Tourette syndrome (TS). METHODS: Serum antibodies against human caudate, putamen, and globus pallidus (interna and externa) were assayed by enzyme-linked immunosorbent assay (ELISA) and Western blot techniques and results were correlated with clinical characteristics and markers of streptococcal infection. SUBJECTS: A total of 41 children with TS (mean age, 11.3 years) and 39 controls (mean age, 12.1 years) were included. RESULTS: Compared with controls, TS subjects had a significant increase in the mean (p=0.006) and median (p=0.002) ELISA optical density (OD) levels of serum antibodies against putamen, but not caudate or globus pallidus. Western blots on 20 control and 20 TS serum samples showed that specific antibodies to caudate/putamen occurred more frequently in TS subjects at 83, 67, and 60 kDa; antigens were present in a synaptosomal fraction. TS subjects with a positive family history of tics had higher OD values (p < or = 0.04), but no association was shown with age of tic onset, tic severity, sudden onset of tics, or presence of attention-deficit hyperactivity disorder or obsessive-compulsive disorder. Risk ratio calculations in TS and control groups and in study subjects dichotomized for high and low putamen OD values were similar for titers of antistreptolysin O > or = 166 or antideoxyribonuclease B > or = 170. A subgroup analysis limited to subjects with elevated streptococcal titers, however, showed a significantly (p < or = 0.004) larger number of TS subjects with elevated OD levels. CONCLUSION: Children and adolescents with TS had significantly higher serum levels of antineuronal antibodies against putamen than did controls, but their relation to clinical characteristics and markers for streptococcal infection remains equivocal.

Adolescent↗

Neuroimmunology of tics and other childhood hyperkinesias.

The etiology of tics and other childhood hyperkinesias is unclear despite attempts to link these movement disorders to neurotransmitter abnormalities and genes. This article will review the studies that suggest that some movement disorders are the result of immunologic factors.

Antibody Formation↗

Abnormalities of the peripheral blood as a presenting feature of immunodeficiency.

The predisposition of immunodeficient patients to the development of peripheral blood abnormalities is well documented. Less often recognized is the presence of immune defects in patients who initially present with "idiopathic" forms of thrombocytopenic purpura or autoimmune hemolytic anemia in the absence of symptomatology indicative of an immuno-deficiency. In this report we describe two children, each of whom had intermittent and at times severe hemolysis and thrombocytopenia secondary to formation of autoantibodies. Although the initial clinical impression was the presence of an "idiopathic" form of platelet or red cell destructive phenomenon, subsequent observations demonstrated abnormalities of the immune system which affected both B and T lymphocytes. In one of the patients there was a marked deficiency of IgA with a moderate but progressive decrease in IgM and IgG. The other patient had a moderate decrease in IgA and a failure to sensitize to dinitrochlorobenzene. Both exhibited dysmorphic features, including small stature, abnormal dentition, hyperelasticity of the skin, and hyperextensibility of the joints. Neither had significant problems with bacterial, fungal, or viral infections. These observations highlight the importance of evaluating the immune system in children with "idiopathic" forms of hemolytic anemia or thormbocytopenia. Demonstration of these abnormalities can have important therapeutic implications and may also provide information regarding the etiology of the blood abnormalities.

Adolescent↗

Pneumocystis carinii pneumonia in children.

From a retrospective study at the University of California, San Francisco, Medical Center, it is evident that pneumocystic carinii pneumonia is being seen more frequently as a secondary complication to the use of immunosuppressive drugs. This disease presents with nonspecific respiratory symptoms, therefore a high degree of suspicion and knowledge of the population at risk are necessary for an early diagnosis. Except for x-ray films of the chest, physical and laboratory studies are of minimal diagnostic value. In a patient with compromised immune defenses and respiratory distress, bilateral diffuse reticular infiltrates seen on a film of the chest are highly suggestive of pneumocystis carinii pneumonia. The diagnosis should be confirmed histologically because a variety of pathogens can cause these findings and each requires a specific treatment. At our institution, open thoracotomy is the method of choice for obtaining a lung biopsy specimen. Pentamidine isothionate is moderately effective against this usually fatal disease, but its effectiveness depends on beginning treatment early in the illness.

Adolescent↗