Does the teddy bear sign predict psychogenic nonepileptic seizures?

This study evaluated whether adults and older teenagers who bring toy stuffed animals to an epilepsy monitoring unit (EMU), i.e., the "teddy bear sign," were more likely to be diagnosed to have psychogenic nonepileptic seizures (PNES) than to have epilepsy. We prospectively evaluated 335 patients, aged 15 years and older, admitted to our EMU over a 19-month period, assessing age at seizure onset, duration of seizures, gender, seizure diagnosis, presence of intellectual disabilities, presence of psychiatric illness, and possession of a toy stuffed animal in the EMU. Among all ages, patients who brought toy stuffed animals were not more likely to have PNES or both PNES and epilepsy than to have epilepsy alone. For those 18 and over, there was a significant difference but only after adjusting for all other patient characteristics, and absolute differences were small. Patients 18 and older with stuffed animals had a 3.21 (95% confidence interval = 1.58, 8.90) times greater odds of being diagnosed to have PNES or both PNES and epilepsy than to have epilepsy alone after adjusting for other patient characteristics (p = 0.022). We conclude that patient possession of toy stuffed animals in the EMU is not a reliable sign of PNES.

Motor stereotypy disorders.

PURPOSE OF REVIEW: This review highlights recent advances in understanding the clinical features, prevalence, and outcomes of motor stereotypy disorders in typically developing children. RECENT FINDINGS: Longitudinal data indicate that stereotypies in children with normal intelligence show an early age of onset, chronicity, and high prevalence of comorbid difficulties, including tics, obsessive-compulsive behaviors, and attention deficit hyperactivity disorder. The underlying abnormality remains unknown, but there is increasing evidence for Mendelian inheritance and a neurobiological mechanism. SUMMARY: Primary motor stereotypies are relatively common in childhood and can be subdivided into three groups (common, head nodding, and complex motor). Movements are similar to those seen in children with autistic spectrum disorders, mental retardation, and sensory deprivation. The role of pharmacotherapy is not established and behavioral therapy can be beneficial.

Pediatric epilepsy syndromes.

BACKGROUND: Pediatric epilepsies are a heterogeneous group of disorders that encompass various epilepsy syndromes ranging from benign to progressive and catastrophic. The past 2 decades have seen major advancements in the appreciation of epilepsy syndromes and their underlying mechanisms. Modern neuroimaging methods have helped to identify structural pathologies and genetic discoveries led to identifying new syndromes and characterization of old syndromes. Epilepsy syndromes are categorized on the basis of a number of features including seizure types, age of onset, clinical features, electroencephalographic expression, and response to treatment. Multiple seizure types, evolution from one type to and another and age-dependent expression are some of the unique features of epilepsy syndromes. The International League Against Epilepsy uses the age-related expression of these syndromes and classifies them by different age groups, allowing easy identification. Early recognition and identification of epilepsy syndromes is essential to provide valuable information on management and prognosis. REVIEW SUMMARY: The purpose of this article is to review the literature data on the prevalence, clinical manifestations, electrographic findings, pathophysiology, prognosis, and treatment options for these syndromes. CONCLUSIONS: Accurate and early diagnosis of epilepsy syndrome, when possible, is essential for both evaluation and treatment because specific antiepileptic therapies are increasingly recognized.

Paroxysmal kinesigenic dyskinesias and pseudohypo-parathyroidism type Ib.

Pseudohypoparathyroidism is a rare cause of paroxysmal dyskinesias. We describe an otherwise well 10-year-old girl who was diagnosed with pseudohypoparathyroidism type Ib after presenting with involuntary movements of the hands and feet that occurred while running or walking. Magnetic resonance imaging of the brain indicated T(1) hyperintensities of the bilateral basal ganglia. A computed tomography scan of the head revealed diffuse cerebral calcifications, most prominent in the basal ganglia. Treatment with calcitriol and calcium carbonate led to a complete resolution of her signs. We recommend that hypoparathyroidism always be considered in patients with kinesigenic paroxysmal dyskinesias, especially insofar as it is a treatable disorder.

Aicardi syndrome mimicking intrauterine hydrocephalus.

Congenital enlargement of the cerebral ventricles is now commonly recognized in utero due to the availability of high resolution prenatal ultrasonography. It is important to distinguish between congenital hydrocephalus and ventricular enlargement due to malformations, infections or other destructive processes because these disorders can have markedly different prognoses. We report an infant diagnosed with Aicardi syndrome in the newborn period based on brain MRI and ophthalmological findings after she was referred for evaluation of hydrocephalus seen on fetal ultrasound. Aicardi syndrome most commonly comes to medical attention because of seizures later in infancy.