Muhammad Athar Javed, Dr Qamar Zaman, Dr Naveed Alam.
Spectrum of acquired demyelinating disorders of the central nervous system in adults in a tertiary care hospital.
Pak J Neurological Sci Jan ;9(1):01-5.

Background: Acquired demyelinating disorders of the central nervous system are diverse group of conditions characterized by destruction or disturbance of myelin sheath in the central nervous system i.e. brain and spinal cord. These vary in their causes, clinical presentations, and prognosis. Objective: To study the spectrum of acquired demyelinating disorders of central nervous systemin adults in a tertiary care hospital of Pakistan. Materials and Methods: This is a retrospective cross sectional descriptive study carried out at department of Neurology, King Edward Medical University/ Mayo hospital,Lahore. Study period was one year from March 2012 till February 2013. Medical records of the patients with history of focal or multifocal neurological deficits suspected of demyelinating disorders were reviewed. Those fulfilling the diagnostic criteria for specific demyelinating disorder were included in the study. Clinical, laboratory and radiological features were recorded on a specific proforma designed for the study. Results were analyzed using SPSS version 17. Results: During this period seventy one patients with various demyelinating disorders were diagnosed. There were 39 (55%) males and 32(45%) females with M: F ratio of 1.2:1.The mean age at presentation was29.5+ 9.3 years. Thirty four (48%) patients were diagnosed as clinically isolated syndrome (CIS), 23(32%) as multiple sclerosis (MS), 8(11.2%) as neuromyelitis optica (NMO), and 2(2.8%) as acute disseminated encephalomyelitis (ADEM). There was one case (1.5%) of each of the following disorders: progressive multifocal leukoencephalopathy (PML); vitamin B12 deficiency with dorsal myelopathy; post cardiac arrest hypoxic/ischemic symmetrical demyelination affecting basal ganglia; and central pontine myelinolysis (CPM).Out of the 34 cases of CIS, 17(50%) cases presented as transverse myelitis, 14(41%) optic neuritis, 2(6%) cerebellitis, and 1(3%) mutlifocal CIS. Among 23 cases of multiple sclerosis, 17(74%) had relapsing and remitting course (RRMS), 4(18%) primary progressive (PPMS), and 2(9%) secondary progressive course (SPMS). Conclusion: Demyelinating disorders are not uncommon in our local population. Inflammatory demyelinating disorders are more common than non-inflammatory causes. Monofocal clinically isolated syndrome (CIS) remained the most common form of inflammatory demyelination especially transverse myelitis followed by optic neuritis. Frequency of relapsing remitting multiple sclerosis was 74% and neuromyelitis optica was 11.2% in our local population. ADEM was rare (< 2.8%) in our adults population.

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