Faculty Bibliography

Autistic disorder and catatonia are neuropsychiatric syndromes defined by impairments in social interaction, communication, and restricted, stereotypical motor routines. Assessments of children with these disorders are typically restricted in scope by the patients' limited ability to comprehend directions. The authors performed systematic assessments of dyskinesias on six prepubertal boys with autistic disorder and mental retardation and on one adolescent male with catatonia to determine if this type of information could be routinely obtained. The boys with autistic disorder had more stereotypies and tics, a greater degree of akathisia and hyperactivity, and more compulsions than the adolescent with catatonia. Catatonia was associated with catalepsy and dystonic postures. The authors conclude that the diagnostic accuracy and specificity of neuropsychiatric syndromes may be enhanced by the systematic assessment of the dyskinesias associated with each condition.

Schizophrenia is a syndrome that is believed to have its onset during very early corticogenesis of the affected patient. The subsequent structural and functional cerebral variations observed in people with schizophrenia suggest an altered overall pattern of brain morphology. The alterations in cerebral structure and function in schizophrenia suggest that an early disturbance of the central nervous sensory modality may be occurring in the patient. Placing the structural and functional cerebral deficits reported in schizophrenia within the context of normal brain structure and function provides a neurological basis to identify early pathological molecular mechanisms and programs that may be resulting in schizophrenia.

A Myoclonus Versus Tic Checklist facilitates making this vexing differential diagnosis. Although it appears to be a valuable tool for clinical, educational, and research purposes, psychometric characterization of the Myoclonus Versus Tic Checklist is needed

Background: Individuals with pervasive developmental disorders are vulnerable to the occurrence of catatonia and persistent functional deterioration. Objective: To facilitate the recognition of the clinical diagnosis of pervasive developmental disorder and catatonia, we describe the manifestations of a patient with a pervasive developmental disorder who exhibited progressive catatonia. Method: A young man with a pervasive developmental disorder who developed progressive catatonia at the age of 14 years was videotaped participating in a structured movement assessment battery over 9 sessions between 16 years 8 months and 19 years 9 months of age. Results: Mutism, social withdrawal, blepharospasm, grimacing, and inability to perform the activities of daily living progressively increase during the course of the illness. Conclusion: Pervasive developmental disorder and catatonia can be diagnosed and monitored in outpatient settings by the use of structured assessment protocols

Autistic disorder presents in early childhood with marked deviations in social interactions and communication and with a restricted and peculiar range of interests and activities. Although probably a heterogeneous condition, autistic disorder includes a class of individuals who also have subtle neurological impediments manifested as disturbances of movement, balance, posture and gait. We review neuromotor and neurobehavioral assessments for persons with autistic disorder and related conditions to improve the classification of the impairments and to facilitate the utilization of therapies specific for each class

Background: Improving the quality of the utilization of psychoactive medication is hindered by the dearth of reliable, valid monitoring procedures. Protocols to assure quality and to foster continuous improvement in the administration of psychoactive medications are needed for clinical, administration, and research purposes. Objective: To develop a simple screening procedure for numbers and dosages of medications to identify serious errors in psychoactive medication administration. Method: The pharmacist provides information about all medications and their dosages taken by every subject in a developmental center for people with mental retardation and developmental disabilities in the past month. The clinical reviewer examines the data to determine if dosages are within the usual therapeutic range. The reviewer also assesses combinations of medications to determine if the subject receives polypharmacy, the administration of two or more medications in the same class. The reviewer and the pharmacist then discuss questionable doses to suggest to the treatment team more effective approaches. Results: This simple protocol can be followed on a monthly bases at large facilities to avoid egregious errors in the use of psychoactive medications. Conclusions: Assessing dosages provides a means to identify serious errors in the administration of psychoactive medications. This procedure is suitable for screening purposes of large numbers of subjects on a daily basis.

LNS is an inborn metabolic disorder characterized by infantile onset of choreoathetosis, dystonia, and stereotypical self-biting. Because it results from an X-linked recessive genetic defect, female cases are rarely encountered. Only 4 such cases have been reported; no discordant twin female case has been previously documented. We report a female patient with classic neurological finding and self-injury. Her KBIT IQ is lower than that of our male cases (male mean IQ=60, female case=39, twin sister=92) Neuropsychological testing in the female case reveals the characteristic impairment of attention, executive functions, free recall, and visual-spatial processing found in male cases. The personality profile in both the male cases and this female case, unstable extravert, is similar but not found in the twin sister. We found reductions in dopamine transporter density (WIN 35,428 binding) of 68% in putamen and 42% in caudate in 6 classic LNS cases with self-injurious behavior (Wong, Harris, et al., 1996). This female patient, the first studied with PET, has a similar reduction in dopamine transporter binding (caudate k3/k4=2.09, putamen k3/k4=l.14.) at the same level as found in male cases. It has been proposed that female LNS cases have nonrandom X-inactivation or double mutations. In this case we propose that X-inactivation preceded twinning and that non-randomness reflects asymmetrical splitting of the inner cell mass. Her mutation has resulted in a female with the features of classic LNS. (NICHD, HD33095)

Clinician safety is a serious concern for health care professionals because of the great risk of violence in health care workplaces, especially emergency and mental health departments. Assaults on mental health staff are often encountered from patients who are intoxicated with, or suffering withdrawal symptoms from, substances. Additionally, violence toward mental health professionals is frequently encountered from patients diagnosed with paranoid schizophrenia, personality disorders, learning disabilities, and mental retardation. Incidents of assault are often ignored by victims and administrators. Administrators must implement several measures to prevent clinician assault

Autistic disorder, an extremely disabling syndrome with onset in early childhood, is associated with multiple comorbid conditions. Although autistic disorder is heterogeneous in its manifestations, there is a subgroup of individuals with autistic disorder who display movements that appear to be unique for the disorders. Hand flapping and a variety of movements termed stereotypies may be pathognomonic of autistic disorder. Therefore, identification of a movement disorder characteristic of autistic disorder may imply that the individual has autistic disorder