Objective: To study the clinical and biochemical features of atypical parkinsonism. Evaluate results of the immune-mediated treatment

Background: Atypical parkinsonism is a heterogeneous group of neurological disorders characterized by parkinsonian features and poor response to levodopa. Early onset of the following additional clinical features can suggests atypical Parkinsonism, this could be dementia, postural instability with frecuent falls, alucinations, eye movement abnormalities, blepharospasm, eyelid apraxia, pyramidal signs, autonomic symptoms like postural hypotension and incontinence, motor apraxia, alien limb phenomenon.

Methods: Patients with atypical parkinsonism and positive antibodies NMDA, LGI1, CASPR2, AMPA, mGluR1, GABA Yo or GAD 65 antibodies were identified retrospectively by review of medical records.All attended between Jan 2014 to dic 2016. Clinical history and examination, MRI CSF and neuroimmunology laboratory evaluations were performed. Paraneoplastic neuronal antibodies (ANNA, AGNA)1, PCA, amphiphysin, CRMP-5, Ma/Ta were identified. Whole body PET CT was performed in 12 patients and limited paraneoplastic work up-chest X-ray, ultrasound abdomen and pelvis was done in others.

Results: 22 atypical parkinsonism (10M/12F) were included. Age ranged from 23 to 75 years (SD — 51.7 ± 9.92 years). Duration of disease ranges from 4 months to 13 years. 4 probable MSA, 2 possible MSA, 1 possible PSP, 4 probable CBS, and 3 had probable PSP. 8 others had parkinsonian features that did not fulfill the criteria of atypical parkinsonism. CSF studies were done in all 22 patients. 16 had abnormal CSF. 16 had pleocytosis, 10 of them had elevated protein ranging from 47.5 to 117.5 mg/dl. Malignancy was seen in only 2 patients. 1 was positive for LGI1 and ovarian cancer, and the other had anti Yo antibodies with uterus cancer. There was a dramatic clinical improvement in terms of UPDRS motor scale (N25% reduction after completion of 5 doses of IVMP). There was an improvement in eye movement, postural instability and cerebellar/autonomic symptoms. 2 had reappearance of symptoms and improvement on restarting steroid. One patient died of infection though there was a good response to treatment.

Conclusions: Atypical parkinsonism could be associated with different antibodies. This theory raises the need to determine these patterns linking these to the prognosis of patients.

References: Dalmau J, Geis C, and Graus F. Autoantibodies To Synaptic Receptors And Neuronal Cell Surface Proteins In Autoimmune Diseases Of The Central Nervous System. Physiol Rev 97: 839–887, 2017. Varley J, Taylor J, Sarosh R. Irani Associate Professor Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK Neuropharmacology xxx (2017) 1e12. Dalmau J, Graus F et al. Antibody-associated CNS syndromes without signs of inflammation in the elderly. Neurology® 2017;89:1–5 JAMA Neurol. 2015;72(11):1304-1312. doi:10.1001/jamaneurol.2015.2378. Balint B, Vincent A, Bhatia et al. Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and Pathophysiology.. doi:10.1093/brain/awx189 BRAIN 2017: Page 1.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/immunological-causes-of-atypical-parkinsonism/