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Título

Gastrointestinal Dysmotility as the Leading Complaints in Sensory Neuronopathy Patients

RESUMO

Case 1:A 43-year-old male patient reported a 2-month history of paresthesia on calves. Examination demonstrated asymmetric loss of vibration and position sense (including pseudoathetosis), Romberg’s sign, diffuse hyporeflexia, and tabetic gait. The patient developed constipation followed by acute abdominal pain. Intestinal pseudo-obstruction was diagnosed, and an exploratory laparotomy was performed but no mechanical obstruction was found. Case 2: A 58-year-old woman reported pain and paresthesia in her right hand initiated 8 years before, with gait imbalance. Neurological evaluation showed diffuse loss of position and vibration sense, Romberg’s sign, and global areflexia. She also reported urinary incontinence and severe constipation with onset in the previous 4 years. HTLV-1 serology was positive. Case 3: A 53-year-old female patient was admitted for investigation of dysphagia associated with post-meal vomiting and paresthesia in the upper limbs for the past 6 months. Neurological examination showed pseudoathetosis and sensory ataxia in the upper limbs, tactile and painful hypoesthesia in the four limbs, diffuse hypopalesthesia, and diffuse hyporeflexia. A diagnosis of achalasia was made and was surgically corrected. NCS and EMG of all three patients demonstrated a severe reduction in SNAPs and preserved motor conduction, compatible with the SN diagnosis. Discussion:The frequency and pattern of dysautonomic features in SN are still poorly understood, despite being remarkably present in clinical practice. A study performed on patients with non-paraneoplastic SN demonstrated the involvement of various subdomains of the autonomic nervous system (ANS), with about half of the patients presenting orthostatic hypotension and three-quarters with cardiovascular autonomic neuropathy. From the 12 cases of intestinal pseudo-obstruction associated with SN reported in literature, only 3 were idiopathic. Autopsy studies of the patients revealed severe damage of myenteric plexus. The patients discussed here differ from the other reports since they are non-paraneoplastic forms of SN. Furthermore, the association between SN and achalasia is also quite rare in the literature, with few cases reported.Final Comments: Gastrointestinal dysautonomia leading to dysmotility is a substantial burden to patients with SN. Understanding the involvement of the ANS in SN is of special interest both for earlier diagnosis of atypical presentations and for improvement in quality of life.