Non-convulsive status epilepticus of frontal origin in mucopolysaccharidosis type II successfully treated with ethosuximide.

At 7 years of age, a female with mucopolysaccharidosis type II (MPS II) showed a sudden deterioration in neurological function, a sleep disorder, and progressive behavioural impairment. Electroencephalography was performed 1 year and 8 months after the onset of the neurological regression and revealed continuous ictal activity in the frontal regions. The female was diagnosed as having frontal non-convulsive status epilepticus. After 5 weeks of therapy with ethosuximide, the ictal electroencephalographic activity disappeared. At the same time, her sleep and cognitive and behavioural functions were observed to improve. This is the first case of MPS type II reported in an individual with non-convulsive status epilepticus. Two main forms of MPS II can be recognized: attenuated and severe. Severe MPS II is characterized by neurodegeneration. No study has explored the relationship between epilepsy and neurological deterioration in MPS II. Our observation shows that epilepsy may be a treatable cause of neurological regression in individuals with MPS II.

A naturalistic study of referred children and adolescents with obsessive-compulsive disorder.

OBJECTIVE: To report on clinical features, comorbidity, and response to pharmacotherapy in children and adolescents with obsessive-compulsive disorder (OCD) naturalistically followed and treated with serotonin reuptake inhibitors (SRIs). METHOD: A consecutive series of 94 patients (65 males, 29 females, age 13.6 +/- 2.8 years), referred in the period January 2001-April 2004, diagnosed with a clinical interview (Diagnostic Interview for Children and Adolescents-Revised), and followed for 10 +/- 6 months, were included in the study. RESULTS: Contamination obsessions and washing rituals were associated with less impairment than other subtypes of OCD. Aggressive sexual obsessions and checking rituals as well as symmetry obsessions and ordering-repeating rituals were more frequently comorbid with tic disorders. According to the Clinical Global Impressions-Improvement scale (score 1 or 2), 63 subjects (67%) were responders to treatment. Nonresponders were more severely impaired and had a higher number of comorbid disorders, namely, bipolar disorder and conduct disorder (p < .05). Forty-seven patients (50%) received an SRI monotherapy, whereas the other 47 (50%) needed other medications. Patients receiving SRI monotherapy were less severely impaired; had a later onset of OCD; were at a younger age at the visit, had higher rates of depression and anxiety and lower rates of bipolar disorder, attention-deficit/hyperactivity disorder, and conduct disorder (p < .05). CONCLUSIONS: Long-term naturalistic prospective studies in pediatric patients with OCD might represent an important source of information for everyday care regarding the effectiveness of a treatment over extended periods of time under routine clinical conditions.

Generalized anxiety disorder in referred children and adolescents.

OBJECTIVE: There are insufficient data on generalized anxiety disorder in children and adolescents. Symptoms and comorbidity of generalized anxiety disorder are described as a function of age, gender, and comorbidity in a consecutive series of referred children and adolescents. METHOD: One hundred fifty-seven outpatients (97 males and 60 females, 50 children and 107 adolescents, age range 7-18 years, mean age 13.4 +/- 2.7 years) were diagnosed as having generalized anxiety disorder, using historical information and a structured clinical interview (Diagnostic Interview for Children and Adolescents-Revised) according to the DSM-IV. RESULTS: Feelings of tension, apprehensive expectations, negative self-image, need for reassurance, irritability, and physical complaints were reported in more than 75% of the participants. Differences in symptomatology according to age and gender were nonsignificant. Depressive disorder was the most frequent comorbidity, being present in 56% of the patients. Comorbid anxiety disorders were present in about 75% of the patients, and 21% showed externalizing disorders. Subjects with comorbid depression had less anxiety comorbidity, subjects with comorbid separation anxiety disorder had higher rates of panic disorder, and subjects with comorbid externalizing disorders had higher rates of bipolar disorder. CONCLUSIONS: Referred children and adolescents with generalized anxiety disorder are heavily symptomatic and have frequent comorbidity. A more precise definition of the clinical picture may help early diagnosis and prevention of superimposed mental disorders.