Compulsive respiratory stereotypies in children with autistic features: Polygraphic recording and treatment with fenfluramine

Abstract

Syncopes due to compulsive respiratory stereotypies were studied in eight patients with autistic features. Most had been referred for episodes thought to be intractable epileptic seizures. Polygraphic recording showed two types of syncope, one induced by prolonged apnea and the other by a prolonged Valsalva maneuver. Fenfluramine, 1.5-3 mg/kg per day, was given in an open trial. In four of five cases with frequent Valsalva maneuvers, respiratory stereotypies and syncopes were suppressed for 2-18 months. Patients with periodic apneas were more severely retarded and had less clear benefit. Side effects consisted of dose-dependent sedation and mild weight loss which stabilized without interrupting treatment. We suggest that these syncopes are volitional and may be associated with pleasant sensations. A double-blind placebo-controlled trial of fenfluramine seems warranted in such patients.

Syncopes and other paroxysmal events

“…EEG/ECG will document the typical changes if an episode is recorded, with ECG showing a combination of tachycardia and reduction of QRS amplitude by about 50% followed by -after about 10 seconds -rebound bradycardia, and a burst of EEG slow activity (Gastaut et al, 1987).…”

Low-dose fenfluramine in the treatment of neurologic disorders: experience in Dravet syndrome

“…Gastaut described the use of fenfluramine in three children with selfinduced syncopes [Gastaut, 1984]. The addition of fenfluramine (30-60 mg/day) led to a disappearance of the syncopes and an amelioration of their autistic behavior.…”

Paroxysmal non-epileptic events

“…As crises de interrupção respiratória podem ter uma aparência assustadora, mas raramente estão associadas a conseqüências sérias; portanto, o tratamento medicamentoso não é indicado 34 . Gastaut et al (1987) descreveram um tipo de síncope que ocorre principalmente em pacientes retardados e/ou psicóticos 35 . Essas síncopes de reflexo auto-induzidas são raras, mas, às vezes, podem causar problemas de difícil diagnóstico, já que podem ser confundidas com ausências ou crises de drop-attacks.…”

“…Às vezes, a perda de tônus postural pode restringir-se ao pescoço ou pode envolver os membros inferiores, causando uma queda. Em alguns casos, a flenfluramina pode ser útil, mas atualmente, devido a sua toxidade cardiovascular, esse medicamento não é usado muito freqüentemente 6,35 . Este padrão é raramente observado em crianças com inteligência normal 6 .…”