Abstract
The gene mutation causing autosomal recessive infantile bilateral striatal necrosis (IBSN) was identified in eight consanguineous Israeli Bedouin families, in a study at Schneider Children’s Medical Center, Petah Tikva, Israel, and other centers.
Highlights
The gene mutation causing autosomal recessive infantile bilateral striatal necrosis (IBSN) was identified in eight consanguineous Israeli Bedouin families, in a study at Schneider Children's Medical Center, Petah Tikva, Israel, and other centers
The paralimbic and sensory association areas involved in tic generation are similar to those implicated in movements triggered internally by unpleasant or emotional sensations, such as pain and itching
The p62 protein is involved in the basal ganglia degeneration
Summary
Event-related functional MRI (fMRI) was used to study the neural basis of spontaneous motor and vocal tics in 10 patients with Tourette syndrome, at the National Institute of Neurological Disorders and Stroke, Bethesda, MD. FMRI activity occurred in sensorimotor areas including suprior parietal lobules and cerebellum. The paralimbic and sensory association areas involved in tic generation are similar to those implicated in movements triggered internally by unpleasant or emotional sensations, such as pain and itching. (Bohlhalter S, Goldfme A, Matteson S et al Neural correlates of tic generation in Tourette syndrome: an event-related functional MRI study. The unpleasant urge and psychic tension that precede and trigger tics are similar to those of pain or itching, and involve the same paralimbic and sensory areas demonstrated by fMRI for tics. The distinction between the neural basis of involuntary motor tics and tics voluntarily acted out to bring momentary relief of the unpleasant sensation needs further study
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