The delayed-onset focal dystonia is a rare sequela of cerebrovascular disease or diffuse cerebral hypoxic damage. The responsible lesion sites for the dystonia are variable and the pathogenesis is uncertain. We describe two children with delayed-onset focal dystonia as a complication of perinatal anoxia. The intervals between hypoxic insult and onset of dystonia were 6 years in one and 3 in the other cases. Our patients did not have a focal lesion; one had scattered white matter lesion and the other had a diffuse frontoparietal atrophy. Delayed-onset dystonia after perinatal anoxia can be also caused by non-focal lesion such as diffuse frontoparietal atrophy or cerebral white matter lesion with long interval delay.
Child ; Dystonia/*etiology ; Female ; Humans ; Hypoxia, Brain/*complications ; Male ; Time Factors

Child ; Dystonia Musculorum Deformans ; diagnosis ; etiology ; genetics ; Humans ; Magnetic Resonance Imaging ; Male

A case of Leigh's disease (subacute necrotizing encephalomyelopathy) is reported with such noteworthy features as early onset, dystonia, paraparesis the presence of low attenuation areas in both basal ganglias on computerized tomography of the brain and the presence of a high signal intensity in both basal ganglias in T2 weighted image by MR. The electron microscopic findings of muscle biopsy are suggestive of pleoconial mitochondrial myopathy.
Basal Ganglia/pathology ; Case Report ; Dystonia/diagnosis/*etiology ; Energy Metabolism ; Human ; Infant ; Leigh Disease/*diagnosis/metabolism/pathology ; Male ; Muscles/pathology

The delayed-onset focal or segmental dystonia is a rare sequelae of cerebrovascular disease. The responsible lesion sites for the dystonia are variable and the pathogenesis is uncertain. This study reports three patients with delayed-onset focal dystonia as a complication of stroke. The interval between hypoxic insult and onset of dystonia were varied from 1 month to 1 year. Two adults and one child had focal lesions at the contralateral basal ganglia. The interval between the brain damage and dystonia did not appear to be related to the age at the time of hypoxic injury. The site of lesions may serve as an important factor in the pathogenesis.
Case Report ; Cerebrovascular Disorders/*complications/diagnosis ; Dystonia/*etiology/physiopathology ; Foot/physiopathology ; Hand/physiopathology ; Human ; Magnetic Resonance Imaging ; Male ; Middle Age ; Tomography, X-Ray Computed

Adult ; Deep Brain Stimulation ; Dystonia ; etiology ; therapy ; Female ; Globus Pallidus ; physiology ; Humans ; Receptors, Dopamine D1 ; physiology ; Subthalamic Nucleus ; physiology ; Ventral Thalamic Nuclei ; physiology

Paroxysmal autonomic instability with dystonia (PAID) appears to be a unique syndrome following brain injury. It can echo many life-threatening conditions, making its early recognition and management a challenge for intensivists. A delay in early recognition and subsequent management may result in increased morbidity, which is preventable in affected patients. Herein, we report the case of a patient who was diagnosed with PAID syndrome following prolonged cardiac arrest, and discuss the pathophysiology, clinical presentation and management of this rare and under-recognised clinical entity.
Adult ; Anxiety ; complications ; Autonomic Nervous System Diseases ; etiology ; Brain Injuries ; etiology ; Critical Care ; Diagnosis, Differential ; Dystonia ; etiology ; Heart Arrest ; complications ; Humans ; Hypoxia ; Male ; Respiration Disorders ; complications ; Syndrome ; Treatment Outcome

PURPOSE: Existing data supports a relationship between pelvic floor dysfunction and lower urinary tract symptoms. We developed a survival model of pelvic floor dysfunction in the rabbit and evaluated cystometric (CMG), electromyographic (EMG) and ambulatory voiding behavior. MATERIALS AND METHODS: Twelve female adult virgin rabbits were housed in metabolic cages to record voiding and defecation. Anesthetized CMG/EMG was performed before and after treatment animals (n=9) received bilateral tetanizing needle stimulation to the pubococcygeous (PC) muscle and controls (n=3) sham needle placement. After 7 days all animals were subjected to tetanizing transvaginal stimulation and CMG/EMG. After 5 days a final CMG/EMG was performed. RESULTS: Of rabbits that underwent needle stimulation 7 of 9 (78%) demonstrated dysfunctional CMG micturition contractions versus 6 of 12 (50%) after transvaginal stimulation. Needle stimulation of the PC musculature resulted in significant changes in: basal CMG pressure, precontraction pressure change, contraction pressure, interval between contractions and postvoid residual; with time to 3rd contraction increased from 38 to 53 minutes (p=0.008 vs. prestimulation). Vaginal noxious stimulation resulted in significant changes in: basal CMG pressure and interval between contractions; with time to 3rd contraction increased from 37 to 46 minutes (p=0.008 vs. prestimulation). Changes in cage parameters were primarily seen after direct needle stimulation. CONCLUSIONS: In a majority of animals, tetanizing electrical stimulation of the rabbit pelvic floor resulted in voiding changes suggestive of pelvic floor dysfunction as characterized by a larger bladder capacity, longer interval between contractions and prolonged contraction duration.
Animals ; Disease Models, Animal ; Dystonia/*etiology ; Electric Stimulation/adverse effects/methods ; Electromyography/methods ; Female ; Muscle Contraction/physiology ; Pelvic Floor/*physiopathology ; Pelvic Floor Disorders/*complications/physiopathology ; Rabbits ; Urinary Bladder/physiopathology ; Urinary Retention/*etiology ; Urination/physiology ; Urine ; Vagina/*physiopathology

Adult ; Anxiety ; diagnosis ; drug therapy ; psychology ; Botulinum Toxins, Type A ; therapeutic use ; Dystonia ; diagnosis ; drug therapy ; etiology ; Hemifacial Spasm ; diagnosis ; drug therapy ; etiology ; Humans ; Male ; Mastication ; Neuromuscular Agents ; therapeutic use ; Risk Factors