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Short report
Rituximab in the treatment of three coexistent neurological autoimmune diseases: chronic inflammatory demyelinating polyradiculoneuropathy, Morvan syndrome and myasthenia gravis Anna Sadnicka,1 Mary M Reilly,1 Cath Mummery,1,2 Sebastian Brandner,1 Nicholas Hirsch,1 Michael P T Lunn1 1
National Hospital for Neurology and Neurosurgery, London, UK Northwick Park Hospital, London, UK
2
Correspondence to Dr Michael P T Lunn, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK;
[email protected] Received 4 May 2009 Revised 12 August 2009 Accepted 27 August 2009 Published Online First 12 May 2010
ABSTRACT A 76-year-old man with a pre-existing diagnosis of myasthenia gravis was admitted to an intensive care unit with pneumonia and type II respiratory failure. In addition, muscle weakness, widespread myokymia, neuropsychiatric disturbance and autonomic disturbance were present. Antivoltage gated potassium channel antibodies, antistriated muscle antibodies and antiacetylcholine receptor antibodies were positive. Nerve-conduction studies demonstrated findings consistent with patchy demyelination. Electromyography confirmed widespread myokymia, and there was evidence of diffuse encephalopathy on electroencephalography. Diagnoses of Morvan syndrome and chronic inflammatory demyelinating polyradiculopathy (CIDP) were made. Treatment with intravenous immunoglobulin, plasma exchange and high-dose steroids were ineffective, and the patient remained dependent on mechanical ventilation. The coexistence of possibly three humorally mediated autoimmune diseases led to treatment with rituximab. Rituximab treatment was followed by an improvement in muscle strength, allowing successful weaning from mechanical ventilation, diminution in myokymia and improved cognition. At follow-up, there was reversal of the neuropsychiatric manifestations and normal muscle strength. This case suggests that rituximab may be useful in the treatment of autoimmune neurological disease refractory to other immunosuppressant therapies. Specifically, it adds further evidence for the use of rituximab in CIDP. As indications for rituximab in humorally mediated disease continue to expand, international multicentre randomised controlled trials are required to prove the effectiveness of this important emerging biological agent.
CASE REPORT A 76-year-old Kuwaiti man was admitted to an intensive care unit (ICU) with type II respiratory failure and an intercurrent chest infection. He had been diagnosed as having myasthenia gravis in Syria 6 years previously on the basis of a history of weakness, positive acetylcholine receptor antibodies (AChR Ab) and response to pyridostigmine therapy. A thymectomy had been performed. Two significant relapses of weakness and a 2-year progressive psychiatric disorder had occurred prior to admission in the UK. 230
On admission to ICU, his eyes were open spontaneously, he intermittently obeyed command, and there was incomprehensible vocal effort. There was a normal funduscopy examination, intact papillary responses to light and blink response to visual threat in all four visual quadrants. There was no evidence of ptosis. Eye movements on dolls eye testing were conjugate with a full range of movement. No facial or palatal asymmetry was noted, and a nasogastric tube was in place for nutrition and hydration. There was myokymia of the facial muscles. He was globally weak, and there was widespread myokymia in all limbs. Tendon jerks were absent, and plantar responses were mute. He displayed evidence of a neuropsychiatric syndrome with reversal of the sleepewake cycle, change in personality, confusion, aggression and emotional lability. There was no history of hallucinations. Autonomic function was affected with several self-terminating episodes of supraventricular and ventricular tachycardia and postural hypotension on sitting. Routine blood tests were unremarkable other than for evidence of his respiratory-tract infection. A cerebrospinal fluid (CSF) analysis was performed twice; the protein concentration was mildly elevated on both occasions (0.6 and 0.5 g/l (reference interval 0.15e0.4 g/l)). We were not able to trace the result of the oligoclonal band analysis from another hospital. CSF white cell counts were
