Transient Improvement of Spinocerebellar Ataxia with Zolpidem

Abstract

to the editor: There is currently no effective pharmacologic treatment for spinocerebellar ataxia. We describe a family of five patients, four of whom had clinical improvement within one hour after the ingestion of zolpidem (10 mg). The diagnosis of spinocerebellar ataxia type 2 was confirmed by molecular analysis. Analysis of DNA for CAG repeat expansions in the SCA1 , 2 , 3 , 6 , and 7 genes revealed expansion of CAG repeats at the SCA2 locus.Patient 1, a 49-year-old man with titubation, dizziness, and loss of balance from the age of 34 years, had deteriorating speech and handwriting. Cerebellar signs included moderate gait ataxia, intention tremor, and dysdiadochokinesis, as well as titubation. Deep-tendon reflexes were all brisk. After treatment with zolpidem, ataxia, intention tremor, and titubation improved moderately. Pollock Park Family Practice Springs 0084, South Africa 1. Salva P, Costa J. Clinical pharmacokinetics and pharmacodynamics of zolpidem: therapeutic implications. Clin Pharmacokinet 1995;29:142-53. 2. Thomas P, Rascle C, Mastain B, Maron M, Vaiva G. Test for catatonia with zolpidem. Lancet 1997;349:702. 3. Clauss RP, Güldenpfennig WM, Nel HW, Sathekge MM, Venkannagari RR. Extraordinary arousal from the semi-comatose state on zolpidem: a case report. S Afr Med J 2000;90:68-72. 4. Cohen L, Chaaban B, Habert M-O. Transient improvement of aphasia with zolpidem. N Engl J Med 2004;350:949-50. 5.Clauss RP, Nel HW. The effect of zolpidem on brain injury and diaschisis as detected by 99mTc HMPAO brain SPECT in humans. Arzneimittelforschung (in press). Correspondence

Zolpidem in a minimally conscious state

“…Subsequent perfusion testing with radiolabelled isotopes showed improved areas of hypoactivity with improved perfusion after zolpidem [8]. Further case reports have been pupblished in blepharospasm, spinocerebellar ataxia and aphasia after stroke where SPECT imaging showed a 40% increase in regional blood flow in Broca's area, left middle frontal and supramarginal gyri and bilateral orbitofrontal cortex [9][10][11]. Zopiclone, a drug in the same class, has also been reported to have the same effect [12].…”

Hypoxic-Ischemic Encephalopathy after Bee Sting and Treatment with Zolpidem: A Case Report

“…However, the exact pathophysiologic mechanism beneath this paradoxic effect is not known. Some authors have suggested that the effects of zolpidem might be associated with the diaschisis phenomenon (16,17,18). Studies using single-photon emission computed tomography, positron emission tomography, and EEG following zolpidem therapy have reported changes in brain activity (prefrontal cortex, thalamus and striatum) (19,20).…”

Nationwide Population-Based Epidemiologic Study on Cerebellar Ataxia in Taiwan

“…Idebenone is more effective than the placebo for halting and reversing the hypertrophic cardiomyopathy associated with Friedreich’s ataxia [25]. Transient improvement in cerebellar symptoms upon treatment with zolpidem was observed in 4 out of 5 family members with spinocerebellar ataxia type 2 [26]. The potential effectiveness of riluzole in symptomatic therapy for diverse forms of cerebellar ataxia was noted in a pilot clinical trial [18].…”