Effect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac disease.
BACKGROUND
Cerebellar syndrome and small fiber neuropathy may complicate celiac disease (CD) and may be resistant to a strict gluten-free diet.


METHODS
Case series.


RESULTS
We report three patients with biopsy-proven CD who developed cerebellar ataxia and neuropathic pain despite strict adherence to a gluten-free diet. A small fiber neuropathy was suggested by skin biopsy findings in two patients. All patients' symptoms, including small fiber neuropathy symptoms, responded to treatment with intravenous immunoglobulin (IVIG). Discontinuation of IVIG in two patients resulted in worsened ataxia that reversed after resumption of IVIG.


CONCLUSIONS
Intravenous immunoglobulin may be effective in treating cerebellar ataxia and small fiber neuropathy associated with CD, suggesting an immune pathogenesis. Further prospective, controlled studies are necessary to determine the long-term response to IVIG or other immunomodulation therapy.
Souayah
N
N
Department of Neurology, New Jersey Medical School, Newark, NJ, USA. souayani@umdnj.edu
Chin
R L
RL
Brannagan
T H
TH
Latov
N
N
Green
P H R
PH
Kokoszka
A
A
Sander
H W
HW
D000328:Adult; D000344:Afferent Pathways / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D002446:Celiac Disease / Q000150:complications*; D002524:Cerebellar Ataxia / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D002531:Cerebellum / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D036421:Nerve Fibers, Unmyelinated / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D009619:Nociceptors / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D010525:Peripheral Nerves / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D020824:Spinocerebellar Tracts / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous