ABSTRACT
Paroxysmal kinesigenic choreoathetosis (PKC) is a neurological condition which results in abnormal involuntary movements that are precipitated by sudden movement. Because of its rarity, large case series of PKC have not been published. We studied 26 patients with PKC, which represents the largest series thus reported. We reviewed our cases with respect to attack characteristics, aetiology, family history, and treatment response. Our population consisted of 23 men and 3 women. Seven patients had a family history of paroxysmal dyskinesia. None of our patients had clear evidence of symptomatic PKC. Two-thirds of our patients had attacks lasting between 30-60 s, and over one-half experienced one to ten attacks per day. Attack distribution varied widely, and most experienced pure dystonia rather than choreodystonic movements. Most patients responded very well to anticonvulsant therapy. We also report the PET results from two of our patients and Bereitschaftspotential abnormalities recorded from two others.
Subject(s)
Athetosis/epidemiology , Chorea/epidemiology , Adolescent , Adult , Age of Onset , Anticonvulsants/therapeutic use , Athetosis/diagnosis , Athetosis/drug therapy , Child , Child, Preschool , Chorea/diagnosis , Chorea/drug therapy , Electroencephalography , Female , Humans , Magnetic Resonance Imaging , Male , Movement , Tomography, Emission-Computed , Tomography, X-Ray Computed , VolitionABSTRACT
We report eight new sporadic cases of paroxysmal dystonia induced by prolonged or sustained exercise and review an additional seven previously reported cases. The attacks in our patients lasted from a few minutes to up to 2 h, and patient age at onset ranged from 2 to 30 years. Four of the eight patients had hemidystonic attacks, both legs were involved in two other cases, and the remaining two patients had involvement of the right foot only. We propose that such cases should be classified as paroxysmal exercise-induced dystonia.
Subject(s)
Dystonia/etiology , Exercise , Adult , Child , Dystonia/diagnosis , Female , Humans , Male , Middle AgedABSTRACT
Focal task-specific tremors besides primary writing tremor have rarely been reported in the literature. We describe nine cases of focal tremors induced by different specific tasks, involving a repetitive and frequently performed movement. Only one of these patients had a family history of any similar disorders. There was no overt dystonia in any of the cases, but these tremors may be forms of focal dystonia, rather than a manifestation of essential tremor.
Subject(s)
Dystonia/physiopathology , Motor Activity/physiology , Motor Skills/physiology , Stereotyped Behavior/physiology , Tremor/physiopathology , Adult , Dystonia/classification , Dystonia/diagnosis , Humans , Male , Middle Aged , Neurologic Examination , Occupational Diseases/classification , Occupational Diseases/diagnosis , Occupational Diseases/physiopathology , Psychomotor Performance/physiology , Tremor/classification , Tremor/diagnosisSubject(s)
Chin , Chromosome Aberrations/genetics , Genes, Dominant/genetics , Spasm/genetics , Tremor/genetics , Adult , Chin/innervation , Chromosome Disorders , Female , Humans , Male , Pedigree , Spasm/diagnosis , Tremor/diagnosisABSTRACT
The sex prevalence of idiopathic focal dystonia is reported from a data base review of all patients seen at the National Hospital of Neurology, Queen Square and King's College, London up to 1993. There was a higher prevalence of females to males in all categories of focal dystonia involving the craniocervical region. The female to male ratio for cranial dystonia was 1.92:1 (P < 0.01) and 1.6:1 (P < 0.001) for spasmodic torticollis. On the other hand, twice as many men than women had writer's cramp (M:F = 2.0:1, P < 0.01). At present, there is no clear explanation to account for this differences in the sex prevalence of different types of focal dystonia.
