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1. The anticonvulsant potency of vigabatrin (gamma-vinyl GABA, GVG) was studied in an open trial in a group of 21 mentally handicapped patients with drug-resistant epilepsy. 2. With this treatment one third of these patients had more than 50% reduction in seizure frequency. The anticonvulsant effect appeared during the first month of therapy and was maintained during a 7-month study. The side effects were mild: mainly tiredness, aggressiveness, and ataxia. Other anticonvulsant drugs remained at baseline levels during GVG therapy. GVG was not found to modulate EEG recordings. 3. According to our results, GVG is effective for treating intractable epilepsy in mentally handicapped patients. [ABSTRACT FROM AUTHOR]

Vigabatrin (gamma-vinyl GABA) is a new anticonvulsive drug that irreversibly inhibits the activity of GABA transaminase. The effect of vigabatrin on neurotransmission-related amino acids in CSF of 28 epileptic patients was studied and the relationship between the amino acid pattern and clinical response during 7 months of administration of vigabatrin. Of this study population, 46% had more than 50% decrease in seizure frequency (responders). In 54% the seizures decreased less than 50% (nonresponders). In the whole study group, the levels of total GABA during vigabatrin treatment were 283%, free GABA 197%, homocarnosine 310% and glycine 128% that of the levels at baseline in the same patients. Glutamate, glutamine, aspartate, asparagine, and taurine concentrations did not change. The amino acid pattern in CSF during administration of vigabatrin did not differ significantly in responders and nonresponders. The study suggests that both GABAergic and glycinergic neurotransmission are affected by vigabatrin. The changes in CSF levels of neurotransmitter amino acids are, however, not necessarily related to the clinical response. [ABSTRACT FROM AUTHOR]

Background: This study addresses the strength of associations between trichotillomania (TTM) and other DSM-IV Axis I conditions in a large sample (n = 2606) enriched for familial obsessive-compulsive disorder (OCD), to inform TTM classification., Methods: We identified participants with TTM in the Johns Hopkins OCD Family Study (153 families) and the OCD Collaborative Genetics Study, a six-site genetic linkage study of OCD (487 families). We used logistic regression (with generalized estimating equations) to assess the strength of associations between TTM and other DSM-IV disorders., Results: TTM had excess comorbidity with a number of conditions from different DSM-IV chapters, including tic disorders, alcohol dependence, mood disorders, anxiety disorders, impulse-control disorders, and bulimia nervosa. However, association strengths (odds ratios) were highest for kleptomania (6.6), pyromania (5.8), OCD (5.6), skin picking disorder (4.4), bulimia nervosa (3.5), and pathological nail biting (3.4)., Conclusions: TTM is comorbid with a number of psychiatric conditions besides OCD, and it is strongly associated with other conditions involving impaired impulse control. Though DSM-5 includes TTM as an OCD-related disorder, its comorbidity pattern also emphasizes the impulsive, appetitive aspects of this condition that may be relevant to classification., (Copyright © 2019. Published by Elsevier Inc.)

Researchers are developing a fertility preservation technique--testicular tissue cryopreservation (TTCP)--for prepubescent boys who may become infertile as a result of their cancer treatment. Although this technique is still in development, some researchers are calling for its widespread use. They argue that if boys do not bank their tissue now, they will be unable to benefit from any therapies that might be developed in the future. There are, however, risks involved with increasing access to an investigational procedure. This article examines four methods of expanding access to TTCP: (1) expansion of institutional review board (IRB)-approved research trials; (2) offering TTCP as an innovative procedure in hospitals; (3) offering TTCP as a standard practice in hospitals; and (4) commercialization of TTCP. The ethical and practical implications of each are evaluated through a comparison with umbilical cord blood banking (UCBB), a technology that has achieved widespread use based on similar claims of future benefit.

The long-term clinical, neurophysiologic, and psychological effects of add-on vigabatrin treatment were evaluated in a group of 36 mentally handicapped patients with drug-refractory epilepsy. After an initial 3-month follow-up period, 15 (42%) of 36 patients had at least a 50% decrease in seizure frequency compared with baseline. After a 2-year follow-up period, nine (25%) of 36 patients retained the initially observed antiepileptic effects of vigabatrin, and after 5 years, eight (22%) of 36 patients did so. Five (33%) of the 15 patients who initially exhibited a favorable antiepileptic response to vigabatrin lost that response during a 5-year follow-up. Partial-onset seizures represented the seizure type best controlled by vigabatrin. Side effects were mostly mild, and plasma levels of other antiepileptic medication remained unchanged. No impairment of psychological performance was observed during vigabatrin treatment compared with baseline. Also, no clear change was observed in the background or epileptiform activity in the electroencephalogram during the study. Our findings suggest that vigabatrin as an add-on therapeutic effectively controls seizures in a subpopulation of patients with severe epilepsy. In addition, the antiepileptic response, if achieved, is long lasting in about half of the patients.

Levels of total gamma-aminobutyric acid (TGABA), free GABA (FGABA), and homocarnosine (HC) were studied in CSF taken from 12 controls and 28 patients with drug-refractory epilepsy before and during 7 months of gamma-vinyl-GABA (GVG) administration. At baseline TGABA and FGABA in CSF of epileptic patients did not differ from that of the controls. In epileptic patients HC was 127% of that in controls. During GVG treatment TGABA was 283%, FGABA 197%, and HC 310% of the levels at baseline in the same patients. The patients who had over 50% reduction in seizure frequency during GVG (responders, 46% of the study population) at baseline had higher TGABA and HC in CSF than patients with less than 50% reduction in seizures (non-responders). During GVG the responders and nonresponders had similar levels of different GABAergic markers. The present study shows that in man GVG treatment effectively suppresses seizures in nearly half of the epileptic patients who had previously been drug-refractory. The elevated levels of GABAergic markers in CSF are not, however, necessarily related to good seizure control during GVG.