Intravenous immunoglobulin therapy for autoantibody-positive cerebellar ataxia.
OBJECTIVE
It has been reported that autoimmune cerebellar ataxias, such as anti-glutamic acid decarboxylase (GAD)-antibody-positive cerebellar ataxia and gluten ataxia, are treatable. Here, we examined the therapeutic efficacy of intravenous immunoglobulin (IVIg) on autoantibody-positive cerebellar ataxia.


METHODS
IVIg therapy was administered in seven autoantibody-positive cerebellar ataxia patients. Therapeutic efficacy was examined in terms of its effects on clinical symptoms and changes in brain perfusion using single photon emission computed tomography (SPECT).


RESULTS
Treatment was effective in four cerebellar cortical atrophy patients (two anti-GAD antibody-positive and two anti-gliadin antibody-positive) and in one anti-thyroid antibody-positive spinocerebellar ataxia type 3 (SCA3) patient, but not in two multiple system atrophy (MSA) patients. All four IVIg effective patients who underwent SPECT showed apparent increases in cerebellar perfusion.


CONCLUSIONS
If cerebellar ataxia with an autoimmune mechanism is suspected and radiological findings do not reveal MSA, it is worth considering immunotherapy including IVIg.
Nanri
Kazunori
K
Department of Neurology, Tokyo Medical University Hachioji Medical Center, Tokyo. synapse@tokyo-med.ac.jp
Okita
Mitsunori
M
Takeguchi
Masafumi
M
Taguchi
Takeshi
T
Ishiko
Tomoko
T
Saito
Hirohiko
H
Otsuka
Takao
T
Mitoma
Hiroshi
H
Koizumi
Kiyoshi
K
D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D002524:Cerebellar Ataxia / Q000000981:diagnostic imaging / Q000276:immunology* / Q000628:therapy*; D003545:Cysteine / Q000031:analogs & derivatives; D005260:Female; D005903:Gliadin / Q000276:immunology; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007453:Iodide Peroxidase / Q000276:immunology; D008297:Male; D008875:Middle Aged; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D005903:Gliadin; D007453:Iodide Peroxidase; D005968:Glutamate Decarboxylase; C059659:technetium Tc 99m bicisate; D003545:Cysteine