Late Onset of Pseudoachalasia in Anti-Hu-Associated Syndrome

TL;DR

A patient with anti-Hu-associated syndrome developed pseudoachalasia nearly 90 months after initial symptoms, highlighting the need for thorough evaluation of new-onset dysphagia in such cases.

Contribution

This case report demonstrates the late onset of pseudoachalasia in anti-Hu-associated syndrome, emphasizing the importance of comprehensive diagnostic evaluation.

Findings

Pseudoachalasia occurred nearly 90 months after the first neurological symptoms in an anti-Hu-positive patient.

Endoscopic botulinum toxin injection provided effective relief after recurrence following pneumatic dilatation.

The patient had a lung carcinoid tumor resected, with no detectable recurrence after surgery.

Abstract

Background: Pseudoachalasia is a rare manifestation of anti-Hu-associated syndrome. We present the case of a 61-year-old female patient presenting primarily with progressive pain and sensory disturbance of all limbs. Neurological symptoms progressed after the primary treatment response and onconeural anti-Hu-antibodies were tested positive, which is often a surrogate to paraneoplastic syndrome. Subsequently, after repeated imaging, a lung carcinoid tumor was resected without detectable recurrence after surgery. Nearly 90 months after the first neurological manifestation, the patient developed dysphagia and the diagnosis of pseudoachalasia was established by esophageal manometry. Due to recurrence after pneumatic dilatation, endoscopic botulinum toxin injection provided good clinical results for the patient.

Purpose: This case illustrates that anti-Hu-associated paraneoplastic pseudoachalasia may occur late in the clinical course, indicating that new-onset dysphagia in anti-Hu-positive individuals should be thoroughly investigated by imaging, endoscopy, manometry, and histology.

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