Dissociative-like Spells in a Child With Neurofibromatosis (type 1)

Abstract

Neurofibromatosis, type 1, (NF1) is a common neurocutaneous disorder of childhood. Little is known about the psychiatric aspects of the condition. We present the case of a 10-year-old male with NF1 and complex spells. For two years he had been experiencing self-limited paroxysms of auditory and visual hallucination, assaultiveness, excited undressing, and amnesia. The spells have been refractory to multiple treatments, including antipsychotic medication. The question remains whether this episodic amnestic disorder is comorbid with NF1 or is caused by it. Neurofibromatosis is a relatively common neurocutaneous disorder that consistently has its onset in childhood. Most common is type 1 (NF1), also known as von Recklinghausen’s neurofibromatosis, which accounts for 85 percent of cases and has an incidence of 1 in 3000 persons (1). Penetrance of NF1 is complete in those with the mutation, whether the mutation is sporadic or familial in origin. Phenotypic expression of the disorder varies greatly, even among family members (2). Clinical features of NF1 are decreased stature, scoliosis, hypertension, cognitive impairment, learning disability, and seizure disorders. Benign and malignant tumors of central nervous system (CNS) and non-CNS origin may occur (3-5). Little is known about the relationship between the disease and psychiatric disorders. Mouridsen and Sorensen (6), in a review of the literature, describe associations between NF1 and autism, learning disability, and attention deficit/hyperactivity disorder (ADHD). In one study, 23 (33%) of 69 adult patients with NF1 had comorbid psychiatric illness: depressive syndromes, anxiety with somatic complaints, and organic