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CASE REPORT
Year : 2006  |  Volume : 9  |  Issue : 1  |  Page : 42-45
 

'Rescue' plasmapheresis in a case of severe steroid unresponsive devic's neuromyelitis optica


Department of Neurology, Sree Chitra Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Correspondence Address:
Sanjeev V Thomas
Department of Neurology, Sree Chitra Institute for Medical Sciences and Technology,Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.22822

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   Abstract 

Neuromyelitis optica (NMO) or Devic's disease is an uncommon idiopathic demyelinating disease of the central nervous system with selective involvement of the optic nerves and spinal cord. There are no scientifically proven guidelines on treatment of this condition either in the acute attacks or on a long-term basis. We present the case of a young girl who presented with the classical features of NMO and who did not show any improvement with intravenous high dose steroid injection, which has traditionally been the first line of treatment in the acute attacks of NMO. She was subjected to 5 cycles of plasmapheresis starting from the 12th day after the last dose of steroids. Patient had dramatic improvement of her symptoms in close temporal relationship with the starting of plasmapheresis. This case highlights the potential role of plasmapheresis as a rescue therapy in the management of steroid unresponsive acute attacks of NMO.


Keywords: Neuromyelitis optica, plasmapheresis, steroid - unresponsive


How to cite this article:
Thomas C, Mini S, Thomas SV. 'Rescue' plasmapheresis in a case of severe steroid unresponsive devic's neuromyelitis optica. Ann Indian Acad Neurol 2006;9:42-5

How to cite this URL:
Thomas C, Mini S, Thomas SV. 'Rescue' plasmapheresis in a case of severe steroid unresponsive devic's neuromyelitis optica. Ann Indian Acad Neurol [serial online] 2006 [cited 2019 Dec 13];9:42-5. Available from: http://www.annalsofian.org/text.asp?2006/9/1/42/22822


Devic's Neuromyelitis optica (NMO) is an uncommon demyelinating disease of the Central Nervous System (CNS), which selectively involves the optic nerves and spinal cord.[1],[2] Although initially described as a monophasic illness, subsequent studies have shown that most of the patients experience a relapsing course.[1] The pathogenesis of the condition is thought to be autoimmune mediated, which is further substantiated by the recent demonstration of a specific serum autoimmune marker, NMO - IgG in upto 70% of these patients.[3] There are no scientifically proven treatment strategies for this condition, but traditionally the mainstay of treatment of the acute attack or the index event has been intravenous high dose steroids. NMO has a poorer prognosis when compared to multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM) and this may reflect the more fulminant and necrotizing nature of the pathology in this condition. There have been increasing reports of cases of NMO being treated successfully with plasmapheresis or IV Immunoglobulin (IVIg) after failure to demonstrate any improvement with IV methyl prednisolone (IVMP).[4],[5] We present the case of a young girl who presented with clinical features of severe NMO and who did not respond to the usual treatment with steroids, but showed dramatic improvement with plasmapheresis.

Case

This 20-year old girl presented with acute painless impairment of vision in both eyes, the right eye being affected more than the left. This progressed initially for two days after which the symptom remained the static. Two days later, she started having ascending paraesthesias of both lower limbs, starting symmetrically in both soles and reaching up to the level of xiphisternum by the 6th day. Thereafter there was no further progression. She noted a 'woody' feeling on touching both lower limbs and had no pain sensation on pinching. Around 3 days after the onset of sensory symptom, she started having painful retention of urine and had to be catheterized. Four days prior to admission, she noted weakness of both lower limbs, which progressed to almost total inability to move the legs. She did not complain of any symptoms in the upper limb. There was no pain in the neck or back. She did not have any symptoms referable to the cranial musculature. She had few episodes of vomiting prior to the onset of neurological symptoms. She presented to us on the 10th day after the onset of symptoms.

Examination revealed a young girl who was alert and cooperative

General physical and systemic examinations were unremarkable. There were no skin, joint or ocular findings suggestive of connective tissue disease or vasculitis. Visual acuity was markedly reduced in the right eye with only finger counting possible at 50 cm. Left eye had a visual acuity of 6/36 on tesing by Snellen's chart. Visual field by confrontation method was normal in the left eye but could not be tested reliably in the right eye. Fundus examination was essentially normal in both eyes except for enlarged optic cups. There was evidence for a relative afferent pupillary defect in the right eye. There were no other abnormalities on cranial nerve examination. Motor system showed both lower limbs to be flaccid with grade zero power in right lower limb and 2/5 power in the left lower limb (using Medical research council power grading). Deep tendon reflexes were brisk in both upper limbs and exaggerated in both lower limbs, with ill sustained ankle clonus bilaterally. Both plantar responses were extensor. Sensory examination showed all primary modalities of sensation to be reduced below D4 spinal segmental level. Upper limbs and neck were having normal power and bulk. There were no cerebellar or meningeal signs and spine examination was unremarkable.

Preliminary investigations including haemogram, peripheral smear and biochemical profile like liver function tests, renal function tests and serum electrolytes were normal. CSF showed 20 lymphocytes per cubic millimeter with a protein of 50 mg/dL and normal glucose. CSF was negative for oligoclonal bands. CSF smear or culture did not grow any bacteria, fungi or tubercle bacilli. CSF VDRL was negative. A work up for systemic vasculitis including Rheumatoid factor, ANA, AntidsDNA, C reactive protein, HIV, HBsAg and VDRL were negative. Manteaux test was negative. MRI of brain was normal except for features of right optic neuritis in the form of increased signal from the right optic nerve and right half of the optic chiasm in the coronal STIR images [Figure - 1]. MRI of spine revealed hyperintense lesion in the spinal cord extending from C6- D7 spinal segments in T2 weighted image, with focal cord swelling in the involved area [Figure - 2]. There was only mild nonhomogenous contrast enhancement. Nerve conduction studies showed normal motor and sensory conduction parameters.

She was treated with IVMP at 20 mg/kg/dose for five consecutive days and was continued on oral prednisolone at 1 mg/kg/day. She did not have any improvement with IVMP immediately or after 11 days of completing treatment. Her lower limb weakness remained the same and visual acuity in both eyes did not show any improvement. She was taken up for plasmapheresis on the 12th day after the last dose of IVMP, which was the 28th day after the onset of symptoms. She was given five sessions of plasmapheresis on alternate days with 25 ml/kg of plasma being exchanged per cycle. Patient started improving in power after the second session of plasma exchange. The improvement in power was sustained and by the end of five sessions of plasma exchange her lower limb power had improved by two grades. She also reported a subjective improvement in vision in both eyes, especially in the right eye, noticed after the third session of plasmapheresis. She was able to walk with assistance two weeks after the plasma exchange. Repeat visual acuity testing showed vision of 6/ 36 in the right eye and 6/18 in the left eye. Her urinary catheter was removed and she started voiding on her own. Two months later, her visual acuity was 6/12 in both eyes and she was fully ambulant without support. The only residual symptom was mild difficulty in getting up from squatting position and sensory dulling below the xiphisternum, the intensity of which is much less.


   Discussion Top


NMO is an uncommon idiopathic demylinating disease of the CNS producing sequential or simultaneous optic neuritis (ON) and transverse myelitis (TM). The patient under reference had presented with features of demyelinating ON and TM satisfied the diagnostic criteria for NMO.[1] The presence of normal brain MRI, spinal cord involvement of more than three segments and absence of CSF oligoclonal bands are points in favour of NMO.[2] Work up for other etiologies for such a presentation including collagen vascular diseases and infective causes were negative.

The prognosis for recovery of vision and muscle power in NMO is often poor when compared to other demyelinating illnesses like ADEM or MS. It is reported that during the first episode of ON in NMO, nearly 40% of affected eyes become completely blind (no light perception) at the nadir of the event.[1],[2] There are no proven treatment strategies and most of the recommended treatments are based on experience of doctors rather than carefully planned scientific trials. Traditionally, the mainstay of management of acute attacks and index events in NMO has been corticosteroids.

This patient who had evidence of severe optic neuritis and myelitis did not respond to the usual course of steroids. However, she showed dramatic improvement after plasmapheresis, in both weakness and visual impairment. A recent double-blind crossover study of plasmapheresis versus sham exchanges documented that plasmapheresis is beneficial in treating exacerbations of demyelinating disease that are not responsive to IVMP, especially in cases of NMO.[4] In another study of steroid unresponsive cases of severe optic neuritis seven out of ten patients showed short term improvement with plasmapheresis.[5] There is reasonable evidence from the limited studies done so far that plasmapheresis may be an effective and relatively safe treatment for steroid unresponsive cases of severe CNS demyelination.[5],[6] Other alternate strategies like IVIg and lymphocytaplasmapheresis have also been shown to be effective in some steroid unresponsive cases of NMO.[7],[8] A combination of steroid plus azathioprine had been tried successfully in reducing the disability and risk of relapse in NMO. Seven newly diagnosed patients with Devic's neuromyelitis optica were treated with long-term prednisone and azathioprine, and were followed every 2 months for at least 18 months. Their Expanded Disability Status Scale score improved significantly (mean at baseline, 9; mean at 18 months, 3; P < 0.005), and no relapses occurred for more than 18 months.[9] NMO often has a more fulminant course than other demyelinating illnesses. It carries the risk of leaving significant residual blindness and weakness. Often there is a need for alternate management strategies particularly when the conventional course of corticosteroids fails to produce any improvement. In an open label trial of Rituximab in eight patients with worsening NMO, it had been shown that treatment was well tolerated and six of eight patients were relapse free. Their median attack rate declined from 2.6 attacks/patient/year to 0 attacks/patient/year ( P =0.0078). Seven of eight patients experienced substantial recovery of neurologic function over 1 year of average follow-up.[10] There is little experience with rituximab in demyelinating diseases and it is not available in India. Use of plamapheresis appears to be more logical since the basic pathogenesis of NMO is thought to be predominantly B cell dysfunction with the presence of circulating autoantibodies. It is unlikely that the improvement in this patient could be due to steroids since the patient did not show any signs of improvement during or until the 11th day after the last dose of steroids. In addition, the close temporal relationship between plasmapheresis and improvement of symptoms is indicative of the efficacy of plasmapheresis in this case. This case has been presented to highlight the role of plasmapheresis as a rescue therapy in steroid unresponsive cases of NMO.Well-controlled multi centric randomized trials need to be undertaken to provide the much needed treatment guidelines for this disabling condition.

 
   References Top

1.Dean M. Wingerchuk, William F. Hogancamp, Peter C. O'Brien, and Brian G. Weinshenker. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology 1999;53:1107.   Back to cited text no. 1    
2.Dean M. Wingerchuk, Brian G. Weinshenker. Neuromyelitis optica: Clinical predictors of a relapsing course and survival. Neurology 2003;60:848-53.  Back to cited text no. 2    
3.Lennon VA, Wingerchunk. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004;364:2106-12.   Back to cited text no. 3    
4.M. Keegan, A. A. Pineda, R. L. McClelland, C. H. Darby, M. Rodriguez, and B. G. Weinshenker. Plasma exchange for severe attacks of CNS demyelination: Predictors of response. Neurology 2002;58:143-46.  Back to cited text no. 4    
5.S. Paus, A. Pr φmse, S. Schmidt, T. Klockgether, Y. Mao-Draayer, and H. Panitch.Treatment of steroid-unresponsive tumefactive demyelinating disease with plasma exchange. Neurology 2003;61:1022-23.  Back to cited text no. 5    
6.K. Ruprecht, E. Klinker, T. Dintelmann, P. Rieckmann, and R. Gold. Plasma exchange for severe optic neuritis: Treatment of 10 patients. Neurology 2004;63:1081-83.  Back to cited text no. 6    
7.J Bakker, L Metz. Devic's neuromyelitis optica treated with intravenous gamma globulin (IVIG). Can J Neurol Sci 2004;31:265-7.  Back to cited text no. 7    
8.AJ Aguilera, TJ Carlow, KJ Smith and TL Simon. Lymphocytaplasmapheresis in Devic's syndrome. Transfusion 1985;25:54-6.  Back to cited text no. 8    
9.Mandler RN, Ahmed W, Dencoff JE. Devic's neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology 1998;51:121920.   Back to cited text no. 9    
10.Cree BA, Lamb S, Morgan K, Chen A, Waubant E, Genain C. An open label study of the effects of rituximab in neuromyelitis optica. Neurology 2005;64:1270-2.  Back to cited text no. 10    


    Figures

[Figure - 1], [Figure - 2]


This article has been cited by
1 Neuromyelitis optica
Jacob, A., Boggild, M.
Annals of Indian Academy of Neurology. 2007; 10(4): 231-239
[Pubmed]



 

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