OBJECTIVES: To present a case of childhood primary dystonia with good response to zolpidem.
INTRODUCTION: Dystonia is a syndrome of sustained muscle contractions causing twisting and repetitive movements or abnormal postures. It is not uncommon in children. Although there are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem, an imidazopyridine agonist with a high affinity to benzodiazepine subtype receptor BZ1, is reported to improve basal ganglia disease including Parkinson'sdisease and various types of dystonia.
THE CASE: 11 year old female with torticollis starting the 7years old. At q10years old symptoms progressed with her back hyperexteded. Her cranial MRI was normal. She was started on Levodopa and carbidopa however no improvement of symptoms was noted. She was started on zolpidem and there was a marked improvement in speech, writing, swallowing, walking and posture observed.
DISCUSSION: Primary dystonia is defined as syndromes in which dystonia is the sole phenotypic manifestation. There is no cognitive decline and cranial MRI is normal. Management of childhood dystonia differs in certain respects from that of adult dystonia. Dopamine responsive dystonia in children is rare, but a trial of L-dopa should be performed on all patients rith childhood-onset dystonia. Dystonia is associated with hypoactivity of the globus pallidus interna, and widespread brain alterations in GABAA/benzodiazepine receptors. Zolpidem binding sites are abundant in the basal ganglia output structure the globus pallidus interna and substantia nigra pars reticulata. It is thought that by binding to these sites, zolpidem help restore Ie ganglionic output influence to the thalamus and motor cortex.
CONCLUSION: There are several options to treat dystonia,its medical treatment is notoriously difficult and often unsuccessful. Zolpidem an imidazopyridine agonist can be given in childhood dystonia unresponsive to dopamine.

Human ; Female ; Child ; Basal Ganglia ; Benzodiazepines ; Carbidopa ; Deglutition ; Dopamine ; Dystonic Disorders ; Globus Pallidus ; Levodopa ; Motor Cortex ; Parkinson Disease ; Pars Reticulata ; Dystonia