Chronic Inflammatory Demyelinating Polyneuropathy During Treatment
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.4.604 on 1 October 1998. Downloaded from 604 J Neurol Neurosurg Psychiatry 1998;65:604–615 degeneration. The protein concentration in 2 Smedley H, Katrak M, Sikora K, et al. CSF was 208 mg/dl, there were no cells. Neurological eVects of recombinant human interferon. BMJ 1983:286;262–4. LETTERS TO Immunoelectrophoresis was normal, and 3 Rutkove SB. An unusual axonal polyneuropathy antiganglioside antibodies (GM1, GD1a, induced by low-dose interferon á-2a. Arch THE EDITOR GD1b, GT1b) were absent. Serum bio- Neurol 1997:54;907–8. chemical studies, including HIV antibody 4 Batocchi AP, Evoli A, Servidei S, et al. Myasthe- nia gravis during interferon á therapy. Neurol- determination, were negative. We ruled out ogy 1995:45;382–3. the presence of cryoglobulins. Although 5 Gorson KC, Allam G, Somovic D, et al. IFN-á was discontinued, the disease contin- Improvement following interferon-á 2A in ued to worsen; the maximal neurological chronic inflammatory demyelinating polyneu- Chronic inflammatory demyelinating ropathy. Neurology 1997:48;777–80. polyneuropathy during treatment with deficit was reached 5 months from onset. The interferon-á patient was given prednisone (60 mg/day) and progressively improved. One year later he Posteroventral pallidotomy can Interferon-á (IFN-á) is widely used for the had no symptoms and showed areflexia only ameliorate attacks of paroxysmal treatment of chronic viral hepatitis. There on neurological examination. A further EMG dystonia induced by exercise have been some reports concerning the showed appreciable improvement. development of autoimmune diseases, par- This is the first report of CIDP develop- Paroxysmal exercise induced dystonia is a ticularly thyroid disease, in patients under ment during treatment with IFN-á. CIDP is rare disorder classified as one of the paroxys- treatment with IFN.1 Disorders including an immune mediated disorder that usually mal dyskinesias.12 In this condition patients autoimmune haemolytic anaemia, pernicious responds to plasma exchange, intravenous develop dystonia, mostly involving their feet, anaemia, thrombocytopenic purpura, sys- gammaglobulin, or corticosteroids, although after prolonged exercise, usually walking or temic lupus erythematosus, Raynaud´s dis- occasionally the disease is refractory to these swimming.1–3 Treatment response is poor to ease, parotiditis, and epididymitis have been therapies. In the past, some authors have both antieplileptic drugs and drugs given for reported. Some neurological problems have reported improvement in patients with CIDP dystonia—for example, anticholinergic also been described2; although most such who were receiving IFN-á.5 The mechanism drugs, muscle relaxants, or acetazolamide.3 adverse events have involved the CNS, several by which IFN induced improvement in these We recently noted the dramatic benefit of cases of peripheral nervous system involve- patients is uncertain, although it may be unilateral pallidotomy in completely abolish- ment have been reported—namely, axonal related to complex immunomodulating ef- ing attacks of paroxysmal exercise induced polyneuropathy,3 neuralgic amyotrophy, mul- fects, possibly by reduction of proinflamma- dystonia of the contralateral foot in one tiple mononeuropathies, and myasthenia tory cytokine concentrations (tumour necro- patient. gravis.4 On the other hand, some authors have sis factor and IFN-ã) which may have a role This 47 year old woman was followed up reported that IFN-á maybeaneVective in the development of inflammatory over 2 years for a 10 year history of attacks of alternative therapy in patients with chronic demyelination.5 The relation between IFN-á dystonia aVecting her right foot, induced by inflammatory demyelinating polyneuropathy and CIDP in our patient is uncertain. exercise. At onset the attacks were mild and (CIDP) who are refractory to conventional Whether IFN-á was the cause of CIDP or were induced by walking long distances. 5 treatments. Two trials using IFN-á and whether their relation was only coincidental During an attack her right foot would invert IFN-â on patients with CIDP are currently in remains unknown. Nevertheless it seems for a few minutes making it diYcult for her to progress. We describe one patient who devel- clear that the treatment mentioned above did continue walking or stand. The attack would oped CIDP during IFN-á treatment. not prevent the development of this demyeli- subside within 2–3 minutes on resting. Two A 29 year old man who had hepatitis C for nating disease with an immunological basis. years after onset the attacks subsided and she 2 years, was started on IFN-á treatment. He IFN-á exerts complex immunomodulator was attack free for 3–4 years. Four years ago had the usual related flu-like syndrome eVects, it can improve or worsen autoinmune the attacks returned and got progressively during the first month of treatment. Previ- diseases. worse, increasing in frequency and intensity. ously he had had some migraine headache Although our findings could be coinciden- Over the past 2 years she could have an attack episodes, but no other medical problems. tal, the data suggest caution, as IFN-á on walking even 10–15 steps. The attacks in After 4 months of treatment, he progressively treatment might yield undesirable eVects the past few years not only made her right developed paraesthesias and weakness in involving autoimmune phenomena. foot to in turn as before but caused her to fall both feet. When he came to our hospital 4 Mª EUGENIA MARZO as the right leg would rise up in the air and http://jnnp.bmj.com/ months later, his condition had worsened. MAR TINTORÉ flex at the knee and hip and there would be Neurological examination disclosed tetra- ORIOL FABREGUES some involvement of the trunk causing her paresia (proximal and distal) with 4/5 XAVIER MONTALBÁN spine to twist to the left. Recently the toes of strength (Medical Research Council scale), AGUSTÍN CODINA the left foot were also noted to curl up during generalised areflexia, and hypoaesthesia both Unit of Clinical Neuroimmunology, Department of attacks. She would never lose consciousness in his hands and feet. EMG data are summa- Neurology, Hospital Vall d’Hebron, Barcelona, Spain and the attacks would last 1–2 minutes and rised in the table. Prolonged distal motor Correspondence to: Dr Xavier Montalban, Unit of then subside. They never occurred in sleep. latencies, slowed conduction velocities, tem- Clinical Neuroimmunology, Department of Neu- Interictally the neurological examination was poral dispersion of the compound muscle rology, Hospital Vall d’Hebron, Psg Vall d’Hebron 119–129, Barcelona 08915, Spain. Fax 0034 3 normal although posturing of the right foot action potentials (CMAPs), marked prolon- on September 25, 2021 by guest. Protected copyright. 4274700; email [email protected] could be induced by repeated prolonged pas- gation of F wave latencies, and a reduction of sive flexion-extension movements of the right sensory and motor CMAPs in both arms and ankle. More recently she also began to have the right sural nerve were found. These find- 1 Burman P, Karlsson A, Oberg K, et al. Autoim- occasional spontaneous attacks. Investiga- ings were consistent with a demyelinating mune thyroid disease in interferon-treated tions including repeated MRI of the head and polyradiculoneuropathy with a mild axonal patients. Lancet 1985:ii;100–1. spine were normal as were tests for Wilson’s disease and other causes of secondary dysto- Nerve conduction studies nia. Examination of CSF gave normal results and disclosed no oligoclonal bands. The Conduction velocity Amplitude (µV (sensory) Distal latency (m/s) (m/s) mV(motor)) patient was negative for the common mito- chondrial mutations. An EMG/nerve con- Sensory: duction study detected no evidence of a Right median 3.1 50 4.2 peripheral neuropathy and somatosensory Right ulnar 2.7 48 1.1 Left sural 2.8 41 8.5 evoked potentials were normal. Polymyogra- Right sural 3 43 3.3 phy confirmed cocontraction of agonists and Motor: antagonist muscle pairs in the right leg during Right median 4.4 34 2.4 an attack supporting an organic basis for the Left posterior tibial 10 39 0.8 dystonia. Surface EEG during an attack and Right common peroneal 7.9 38 3.6 interictally disclosed no abnormality. The F wave: Latency F-M Incidence (n (%)) patient was tried on a variety of treatments Right median 42.9 30 (100) including baclofen, levodopa, benzexhol, Right ulnar 36.3 31 (60) tetrabenazine, and acetazolamide without Left posterior tibial 65.5 57 (70) Right common peroneal 63.7 57 (70) benefit. DiVerent antiepileptic drugs given individually or in combination (1g sodium J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.4.604 on 1 October 1998. Downloaded from Letters, Correspondence, Book reviews 605 valproate with 1 g carbamazepine, and 2 m Sudden appearence of invalidating torsional dystonias were still present and clonazepam a day) also did not help. The dyskinesia-dystonia and oV fluctuations rated 10 (SD 2). Bromocriptine up to 10 failure of the medical treatment and the after the introduction of levodopa in two mg/day was not tolerated. During the next 2 frequency of up to 10 or more attacks a day dopaminomimetic drug naive patients years levodopa treatment could not be increased. His UPDRS scores was 89 at 2 made normal functioning impossible for the with stage IV Parkinson’s disease patient and therefore a surgical option was weeks before his sudden death due to appar- considered particularly as the attacks were ent cardiovascular complications with cardiac mainly unilateral. With the consent of the Hyperkinesias (dystonia, dyskinesia) are, with arrest. A postmortem examination showed patient a left posteroventral medial palli- fluctuating akinesias, the most debilitating anteroinferior myocardial infarction, and dotomy was carried out as in a previously disturbances appearing during the advanced normal brain structures with depigmentation 1 of the nigral structure.