MSA Mimic? Rare Occurrence of Anti-Hu Autonomic Failure and Thymoma in a Patient with Parkinsonism: Case Report and Literature Review

Abstract

Thymoma is a tumor originating from thymic gland, frequently manifesting with paraneoplastic neurological disorders. Its association with paraneoplastic dysautonomia is relatively uncommon. Here, we describe the challenging case of a 71 year-old female who developed subacute autonomic failure with digestive pseudo-obstruction, dysphagia, urinary tract dysfunction and orthostatic hypotension complicating an underlying extrapyramidal syndrome that had started 3 months before hospital admission. Autonomic symptoms had 2-month course and acutely worsened just before and during hospitalization. Combination of severe dysautonomia and parkinsonism mimicked rapidly progressing multiple system atrophy. However, diagnostic exams showed thymic tumor with positive anti-Hu antibodies on both serum and cerebrospinal fluid. Complete response of dysautonomia to immunoglobulins followed by thymectomy confirmed the diagnosis of anti-Hu-related paraneoplastic neurological syndrome. With regards to extrapyramidal symptoms, despite previous descriptions of paraneoplastic parkinsonism caused by other antineuronal antibodies, in our case no relation between anti-Hu and parkinsonism could be identified. A literature review of published reports describing anti-Hu positivity in thymic neoplasms highlighted that a definite autonomic disease due to anti-Hu antibodies is extremely rare in patients with thymoma but without myasthenia gravis, with only one case published so far.

Orthostatic Hypotension and Albuminocytologic Dissociation as Primary Manifestations of the Paraneoplastic Syndrome

“…In our cases, OH was the predominant symptom without peripheral neuropathy such as obvious numbness or weakness. In most published cases with positive anti-Hu antibody and OH, patients were usually diagnosed as small cell lung cancer or other obstruction before or together with OH [11][12][13]. But in our cases, the diagnosis of small cell lung cancer appeared at half to 1 year after the onset of OH although contrast lung CT was performed repeatedly.…”

Parkinsonism and dysautonomia with anti-CV2/CRMP5 associated paraneoplastic neurological syndromes mimicking multiple system atrophy: a case report

“…It has to be emphasized that immunotherapy leads to a better prognosis, thereby the possibility of such diseases should be considered preferentially. 5) PNS [ 11 ]. Older patients showed rapid progression and risk factors associated with tumors.…”

Multiple system atrophy

“…Over the last few years, there have been several case reports of autoimmune antibody mediated disease (CV2/CRMP5, Anti-Hu, Homer-3) causing symptoms in keeping with MSA. [7][8][9] However, these cases usually stand out from typical MSA in having with extremely rapid symptom progression, within weeks to months.…”