Dystonia: case series of twenty two patients.
OBJECTIVE
Dystonia is a rare neurological disorder characterised by sustained muscle contraction with resultant bizarre muscle movements and hence bizarre posturing. The pathophysiology is not well understood but it is believed there is dysfunction of the cortico-striatal-thalamo-cortical circuitry and projections. There is very scanty literature in sub-Saharan Africa.


METHODS
Retrospective descriptive case series.


METHODS
Outpatient clinics of the authors, the Nairobi Hospital and the Mater Hospital, Nairobi, Kenya.


METHODS
A cohort of twenty two consecutive patients of indigenous Kenyan origin, seen by the authors between 1996 and January 2005 were described. This study was carried out to describe characteristics of 22 patients.


RESULTS
Males comprised of 15 (68.2%) of the cases. Late onset dystonia was found in 12 (54.5%) cases. Over half of the patients had focal dystonia and only 13.6% had generalized dystonia, all of whom were of early onset variety. There was significant delay in diagnosis of dystonia with 68.2% of patients being diagnosed after 12 months of onset of symptoms.


CONCLUSIONS
Dystonia is rare; however, multi-centre studies are required to map out prevalence rates of the disease in the country. There is need to highlight the presentation of the disease amongst clinicians and patients as this would improve diagnosis rate, early intervention and appropriate management. Genetic studies need to be done.
Jowi
J O
JO
Kenyatta National Hospital, P.O. Box 19624-90202, Nairobi, Kenya.
Musoke
S S
SS
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001905:Botulinum Toxins; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D046690:Deep Brain Stimulation; D020820:Dyskinesias / Q000209:etiology; D004421:Dystonia / Q000209:etiology; D020821:Dystonic Disorders / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007630:Kenya; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors
D001905:Botulinum Toxins