A case of Tourette’s syndrome in association with neurofibromatosis 1 is reported in an 11-year-old boy evaluated at the Instituto de Ciencias Neurologicas, Lima, Peru. Tics began at 5 years, manifested by excessive blinking, and followed by head nodding and shoulder shrugging. At 6 years of age, he developed symptoms of ADHD and learning problems, leading to academic underachievement. At 8 years of age, tic vocalizations began, and at 10 years, a painless intranasal tumor developed that caused deformity of the nasal cartilage. Examination revealed multiple cafe-au-lait spots. Biopsy of the nasal tumor revealed a neurofibroma. MRI showed hyperintensities (T2-weighted) and hypointensities (T1-weighted) in both pallidi. Head CT was normal. [1]
COMMENT. This may be the first documented case of an association between neurofibromatosis 1 and Tourette’s syndrome.
Hemolytic streptococcal infection as a precipitant of tics was evaluated at Johns Hopkins University, Baltimore, MD [2]. Of 80 consecutive children with a diagnosis of tic disorder, 53% had a sudden onset or worsening of tics, and in 15 of these 42, the exacerbation was associated with infection, streptococcal in 9. Approximately 11% of children with tic disorders have a worsening of symptoms within 6 weeks after a streptococcal infection. A relationship of strep infection to tic disorders continues to unfold.