Autoimmune Neurological Diseases - Med

1 JMAJ, September 2004 Vol. 47, No. 9425 This article is a revised English version of a paper originally published inthe Journal of the Japan Medical Association (Vol. 129, No. 7, 2003, pages 921 925).IntroductionThis paper describes some representativeautoimmune Neurological Diseases . As describedlater, the common types of the Diseases inJapan are different from those in Westerncountries; for example, the optic-spinal formof multiple sclerosis and the axonal form ofGuillain-Barr syndrome are more common inJapan than in Western countries.

2 This suggeststhat the development of Autoimmune neuro-logical Diseases varies among races and issubstantially influenced by environmental fac-tors. Therefore, care is needed in interpretingmedical textbooks and literature published inWestern Neurological DiseasesJMAJ 47(9): 425 430, 2004 Fumihito YOSHII* and Yukito SHINOHARA**Associate Professor, **Professor,Department of Neurology, Tokai University School of MedicineAbstract:Multiple sclerosis, myasthenia gravis, and Guillain-Barr syndromeare Neurological Diseases induced by abnormal autoimmunity.

3 Since these dis-eases show characteristic clinical courses and Neurological symptoms, they can bediagnosed with appropriate examinations. However, it is necessary to note that thecommon types of these Diseases in Japan are not always the same as those inWestern countries. Based on an increasing knowledge of the pathological mecha-nisms of the Diseases , various immune therapies are being words: Autoimmune Neurological Diseases ; Multiple sclerosis;Myasthenia gravis; Guillain-Barr syndromeMultiple SclerosisThe diagnosis of multiple sclerosis (MS) ismade according to the conventional clinicaldescription of spatially and temporally sep-arated multifocal central nervous lesions.

4 1)Pathologically, MS presents perivascular in-flammation and myelin sheath destruction(demyelination plaques) in the white prevalence of the disease in Caucasiansis 40 to 100 per 100,000 individuals, while it isonly about one-tenth of this figure among theJapanese. The disease type is also differentbetween Caucasians and Japanese the cere-bral and optic-spinal forms are predominant inthe former and the latter, respectively. Autoimmune Diseases426 JMAJ, September 2004 Vol. 47, No. 9 The examination of evoked potential isuseful for detecting clinically asymptomaticpotential lesions that cannot be read in MRimages.

5 It is performed for visual, auditory, andsomatic sensation when any of the sensorypathways is abnormal, its signal latent time Clinical courseMS mainly develops in patients in theirtwenties to fifties, with a peak being notedbetween 25 and 30 years. Most patients areclassified into relapsing/remitting MS in whichperiods of exacerbation and periods of stableor improved clinical state occur may recur from several times a yearto only once every several progressive MS that progresseschronically from the onset has been reported inabout 10 to 15% of Western patients,2) but onlyin 5% of Japanese patients.

6 This form of MS hasto be differentiated from neurodegenerativediseases or cerebral about 10% of patients with relapsing/remitting MS, the disease gradually becomesprogressive within 10 years (secondary pro-gressive MS). Although this form of MS isobserved in 20 to 40% of patients in Westerncountries, it is less frequently observed Neurological symptomsMS has a large variety of Neurological symp-toms. It often occurs with vision disorder oracute transverse myelitis in Japanese becausemany Japanese MS patients are classified intooptic-spinal MS.

7 The symptoms that maydevelop during the clinical course of MSinclude reduced vision, double vision, dys-kinesia/paresthesia, spasm, dysuria, ataxia,tremor, cognitive dysfunction, and Lhermitte ssign (an electric shock-like sensation runningfrom the back to the limbs when the neck isbent forward, and associated with lesions in thecervical-spinal cord posterior funiculus). Inaddition, paroxysmal symptoms may occur,F. YOSHII and Y. SHINOHARA1. Diagnostic examinationMRI shows demyelination plaques in thewhite matter around the cerebral ventricles,brain stem, cerebellum, and spinal cord as highsignal intensity areas in T2-weighted or fluidattenuated inversion recovery (FLAIR) images(Fig.)

8 1). However, attention should be paid tothe fact that similar findings may also beobserved in patients with other inflammatorydiseases, ischemia, metabolic Diseases , fluid examination shows in-creased IgG in about 70% of the patients, witha resultant increase of the IgG index (obtainedby dividing the ratio of IgG to albumin in thecerebrospinal fluid by the corresponding ratioin the serum). The positive rate of oligoclonalbands of about 35 to 46% in Japanese patientsis lower than in Westerners. Furthermore,although the number of cells in the cerebro-spinal fluid of MS patients should be less than50/mm3, the cell numbers exceed 50/mm3 in aconsiderable number of Japanese 1 Head MRI (FLAIR) image of a patient withmultiple sclerosis: multiple high-density areasare observed in the white matter around thelateral , September 2004 Vol.

9 47, No. 9427 Autoimmune Neurological Diseases including trigeminal neuralgia, paroxysmalarticulation disorders, and dystonic paroxysm(often called tonic convulsions).4. TreatmentAdrenocortical steroids have long been usedfor the treatment of relapsing/remitting most reliable therapy is the intravenousdrip infusion of 1,000 mg of methylpredniso-lone (for 3 days), followed by the oral admin-istration of prednisolone. Although these ster-oids shorten the periods of administration orsymptomatic relapse, they do not reduce therelapse rate or change the long-term may be effective for patientswho do not respond to a massive dose and selective serotonin reup-take inhibitors serve as useful symptomatictreatment for relieving fatigue.

10 Carbamazepineis often effective for trigeminal neuralgia andLhermitte s sign. Carbamazepine or phenytoinis used to treat dystonic -1b (Betaferon ) was approvedin Japan in September 2000 for relapsing/remitting MS and secondary progressive MS toreduce the relapse rate. It is also effective foroptic-spinal MS. It is used by subcutaneouslyinjecting 8 MIU (million international units)every other day. It has been reported to causeadverse effects such as influenza-like symp-toms, redness/pain/necrosis at the injectionsite, and GravisThe prevalence rate of myasthenia gravis(MG) has been reported to be 5 to 7 per100,000 people.