Clinical features, role of mobile plasmapharesis unit and outcome in patients with acute inflammatory demyelinating polyradiculoneuropathy [Guillain-Barr‚ syndrome]

To study the clinical features, role of mobile plasmapharesis unit and outcome in patients with acute inflammatory demyelinating polyradiculoneuropathy [AIDP]. Retrospective Cross Sectional Study. This study was conducted at Neurology Department, KEMU, Lahore from July 2008 till June 2012. Patients from various hospitals [both public and private] fulfilling the Ashbury and Cornblath's Clinical Diagnostic Criteria for GBS and requiring plasmapharesis were included in the study. For this purpose a special proforma was designed to be filled by the primary physician at the time of request for mobile plasmapharesis service. This service was provided by a donor organization namely Pakistan Myasthenia Gravis Welfare Organization [PMWO] based at a public hospital in Lahore. Plasmapharesis was started according to the guidelines, as soon as possible after admission, if patient had history of progressive weakness. The protocol of this treatment was to exchange 200 to 250 ml of plasma per kilogram of body weight in five sessions within 7 to 10 days. The replacement fluids most often consist of 0.9% normal saline, haemaccel and/or albumin. Recovery was assessed by modified Hughes Guillain-Barr‚ syndrome disability scale. A total of 152 patients were included in the study with 94 [61.8%] males and 58 [38.2%] females and M: F ratio of 1.62:1. The mean age was 32.66 [SD 15.89] with range from 7-80 years. One hundred and nine [72%] cases presented between 11-40 years of age. All patients were treated with five sessions of plasmapharesis. Drop out rate for plasmapharesis was 1.5% implying its good tolerability. Out of the total of 152 cases, 149 [98%] cases presented with progressive areflexic weakness and 3 [2%] patients with bilateral external Ophthalmoplegia, areflexia and ataxia [Miller-Fisher variant]. Sensory symptoms were present in 31[20.4%], bulbar weakness in 29[19.1%], and bilateral facial weakness in 25[16.4%] cases. Severe respiratory distress requiring ventilatory support occurred in 36[23.7%] cases. Pearson's correlation revealed that gender and age were not risk factors for the development of ventilatory failure [p=0.354; 0.803], bilateral facial weakness [p = 0.121; 0.473] or bulbar weakness [p= 0.383; 0.745] respectively. Overall mortality was 5% and all these cases developed severe respiratory distress and needed ventilatory support. Complete recovery occurred in 90% cases and 5% had residual deficit [Hughes disability scale severity 1 and 2] at mean follow up of six months. Our study showed that GBS is statistically more frequent in males than females in our local population with maximum frequency between 11-40 years of age range. However, the two factors i.e. gender and age has no significant association with the development of ventilatory failure, bilateral facial weakness and bulbar weakness. Areflexic motor weakness was the commonest presenting feature. Plasmapharesis remained very effective therapeutic option which is cheaper and affordable in our poor socio-economic setting. Mobile unit service provided an excellent opportunity to treat most of these patients at their native hospitals. We recommend that government and donor organizations should develop more mobile plasmapharesis services in all major cities which can cover nearby district and tehsil hospitals

Chronic relapsing inflammatory optic neuropathy [Crion]

This newly recognized entity named chronic relapsing inflammatory optic neuropathy [CRION] is a form of inflammatory optic neuropathy which is frequently bilateral and often painful, and is characterized by relapses and remissions. There is no evidence of acquired demyelinating disorders and systemic collagen vascular or granulomatous diseases. To study the clinical features and response to treatment in patients with recurrent optic neuritis consistent with diagnosis of chronic relapsing inflammatory optic neuropathy [CRION]. Materials and. This is retrospective cross sectional study carried out at department of Neurology, King Edward Medical University, Lahore. Patients with history of recurrent episodes of acute or subacute loss of vision accompanied by pain consistent with optic neuritis, unilateral or bilateral, but, without evidence of an acquired demyelinating disorders, systemic collagen vascular orgranulomatous diseases were included in the study. The response to various treatments was also analyzed.A total of 4 patients were identified. All were females with mean age at presentation 35.50+8.10 years and mean age at onset of first episode was 28+10.92 years. Mean duration of illness was 7. 25 + 4.57 years. The number of episodes varied from 3-6 [mean 4+1.41episodes]. Mean episodes of right optic neuritis were 2.25+0.95 and left side 1.50+ 0.57. Only one patient had an episode of simultaneous bilateral optic neuritis. All patients experienced severe pain with loss of vision to finger counting at less than one meter. MRI brains, detailed vasculitis profile, serum angiotensin converting enzyme [ACE] inhibitor level, and X-ray chests were normal in all patients. The CSF for oligoclonal band was also negative. All patients received pulse therapy with methylprednisolone 1 gm daily for three days followed by two weeks oral taper. Most patients improved after first episode but there was partial recovery after the second and subsequent episodes. Only one patient had complete loss of vision in one eye. Two patients received long term oral steroids and azathioprine with prevention of further relapses. One patient received beta interferonwith complete remission so far. One patient received cyclosporine and oral steroids with remission for 6 years but subsequently relapsed after immunotherapy was discontinued. Chronic relapsing inflammatory optic neuropathy [CRION] should be considered in patients with history of recurrent optic neuritis after exclusion of acquired demyelinating disorders, and systemic collagen vascular or granulomatous diseases