Objective: To illustrate an unusual clinical presentation of Parkinson´s disease (PD) that was effectively treated with immune adsorption.

Background: Autoimmune mediated movement disorders may be open to causal treatment options; as such early correct diagnosis is essential. ITPR 1 antibodies have been described in four cases with autoimmune cerebellar ataxia, however, their pathogenicity is still unclear¹. ITPR 1 gene mutations have been identified in recessive ataxia forms, SCA 15 and SCA 29 ² . Here we report on a case of ITPR 1 antibodies present in a PD patient that was subsequently successfully treated by immune adsorption.

Methods: Retrospective chart review and clinical examination of the patient.

Results: This 63 year old active man was first diagnosed with Parkinson´s disease in 2003 presenting with unilateral left-sided resting tremor, slight left-sided rigidity and bradykinesia. DAT scan revealed severe bilateral reduction of striatal dopaminergic uptake, more prominent on the right side. Subsequently, the disease progressed slowly over time and showed responsiveness to levodopa.

In 01/2016 the patient rapidly developed progressive visual hallucinations without insight, delusional jealousy and cognitive impairment. Within a few days the patient displayed decreasing levels of consciousness and was admitted to the intermediate care unit.

Magnetic resonance imaging showed slight diffuse supratentorial atrophy and the EEG indicated severe encephalopathy. Cerebrospinal fluid analysis revealed high levels of ITPR1 antibodies (1:320), an antineuronal antibody of the IgG1 subclass type that is known to bind to Purkinje cell somata and dendrites¹. The first add on treatment approach with intravenous immunoglobulin showed no clinical effect. Following repeat immune adsorption, serum antibody levels decreased from 1:1000 to 1:320 to 1:100, along with clinical improvement (cognition, psychopathology, motor symptoms).

Conclusions: Our case report demonstrates that reducing antineuronal antibodies in a PD patient by immune adsorption may be an effective therapy.

References: ¹ Jarius S. et al. J Neuroinflammation 2014

²  Parolin Schneckenberg R. et al. Brain 2015

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MDS Abstracts - https://www.mdsabstracts.org/abstract/parkinson-syndrome-associated-with-inositol-145-triphosphate-receptor-antigen-type-1-itpr1-antibodies/