Objective:  Evaluate the gender,mean age,clinical spectrum and non–motor symptoms of the disease in Pakistan,a developing country of South East Asia.

Background: Dystonia,a neurological syndrome,is characterized by stereotypic,abnormal muscle contractions and disabling movements.Developed countries have enough data for its incidence and prevalence,often relating it to severe morbidity and mortality,but for developing countries like Pakistan scarce data is available.

Methods: This one year study (May 2015 to October 2016), was conducted in local neurology clinic visited by many referred patients from adjacent cities and provinces because of better health care facilities.Informed written consent was obtained from all patient or their guardians for the documentation and evaluation of clinical features including motor and non-motor symptoms along with demographic distribution, laboratory,radiological investigation,medication and response towards medication

Results: Excluding secondary cases,52 patients were observed with 41 male and 11 female. Mean age of onset of disease was 42.Primary distribution varies from 45% cervical dystonia, 19% Blepharospasm,12% Oromandibular dystonia,5% Writer’s cramp to 2% Lower limb dystonia.Four patients(5.7%) have positive family history with one parental consanguineous marriage. Among 52 patients, 36(69%) belong to rural area and 16(30.7%) have urban residence. Magnetic resonance imaging and computed tomography revealed no significant Basal ganglia calcifications and in 5 cases aging was a major contributor for age related cerebral atrophy leading to Dystonia.Red blood cell morphology was normal for 86%. Bone profile (75%) exhibited below average serum calcium level (< 2.3mmol/l.) and those patients upon the administration of Vitamin D felt improvement in non-motor symptoms like depression and fatigue. Most effective treatment were Levodopa,Tetrabenazine and Botax(Botulinum toxin A). Among those 09 persons have dopa-responsive dystonia and 21 were given Botax.Depression(71%),lack of self-confidence(81%),insomnia(58%),fatigue(33%) and dizziness(39%) are some common non-motor symptoms observed by the patients from the time of onset of disease.

Conclusions: Our study adds valuable data to existing literature for better diagnosis and treatment.Dystonia,more prevalent among males and cervical dystonia is most common type of dystonia.Dominating factors of rural area,leading to Dystonia needs further investigation.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/prevelanceclinical-pathology-and-treatment-of-dystonia-in-pakistani-population/