Progressive delayed-onset dystonia after cerebral anoxic insult in adults.
The basal ganglia, especially the globi pallidi (GP), are highly vulnerable to generalized cerebral anoxia/hypoxia. We report on 2 new cases with delayed-onset generalized dystonia due to cerebral anoxia. The onset of dystonia in both of our patients was delayed by about 2 months. In both cases, the unusual feature was the progressive worsening and the spread of dystonia over many years after delayed onset. Dystonia progressed for 16 years in Case 1 and for 4 years in Case 2. Furthermore, initial magnetic resonance imaging (MRI) scan of Case 1 showed mild changes of the internal capsule sparing the basal ganglia. Years later, in line with clinical progression, the follow-up MRI scan showed isolated bilateral lesions involving the entire GP. MRI scans in Case 2 showed bilateral lesions of caudate and lentiform nuclei. There may be several mechanisms underlying delayed and progressive symptoms after time-limited brain anoxia. We hypothesize that anoxia-induced excitotoxicity resulting in mitochondrial dysfunction and subsequent apoptosis may explain, at least partly, the delayed-onset and progressive extrapyramidal syndromes seen in these patients.
Kuoppamäki
Mikko
M
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, United Kingdom.
Bhatia
Kailash P
KP
Quinn
Niall
N
D000328:Adult; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000209:etiology*; D018450:Disease Progression; D020821:Dystonic Disorders / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000473:pathology; D006323:Heart Arrest / Q000150:complications; D006801:Humans; D002534:Hypoxia, Brain / Q000150:complications* / Q000175:diagnosis; D020772:Internal Capsule / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination