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Título

Anti-Hu syndrome with simultaneous motor, sensitive and autonomic neuronopathy: a case report

RESUMO

Case presentation
A 67 year-old male with a medical history of prostate cancer, with no evidence of active disease in the last year, started numbness in the right leg and left hand in May 2019, which progressed to paresthesias and paresis in the four limbs (asymmetrical, worse in MSE), reporting difficulty picking up objects, writing and walking. In July 2020, he began to present episodes of seizures reported as a cephalic version, automatisms and unmotivated laughter. Neurological examination showed diffuse paresthesia and areflexia with diminished vibration and proprioception sensitivity in addition to sensory ataxia. Also, prominent and asymmetric weakness was evidenced on the left hand, with signs of muscular atrophy and grade two strength. Pupils were symmetric with no photomotor response. Nerve conduction study and electromyography showed absence of diffuse sensitive responses and chronic motor commitment of left C8-T1 roots, suggesting motor root or anterior horn lesion. Brain MRI was normal, and spine axial T2-weighted sequences showed hyperintense lesions in the dorsal columns, compatible with sensory ganglionopathy. Further investigation revealed positive serum anti-Hu antibodies. Neoplastic screening with chest and abdomen CT and whole-body PET-CT were negative. The patient was treated with intravenous methylprednisolone 1g/d per five days during three consecutive months, intravenous cyclophosphamide for three months, and five plasmapheresis sessions for each month during two consecutive months, but showed no improvement. In the follow-up, patient presented constipation and PET-CT showed important gastroparesis. New neoplasia was not identified until two years after the first symptoms.

Discussion
Anti-Hu-syndrome occurs when there is a dysfunction of the peripheral, autonomic and central nervous systems caused by an immune disorder. Small cell lung cancer is found in more than 90% of cancer. Anti-Hu syndrome may present with neuropathy, ganglionopathy, Iimbic encephalitis, brainstem encephalopathy, opsoclonus-myoclo¬nus, paraneoplastic cerebellar degeneration or myelopathy. We report a case of anti-Hu syndrome presenting simultaneous evidence of motor, sensitive and autonomic neuropathy, with no neoplasia identified. Motor commitment was prominent with extension of the lesion to anterior horn.

Final Comments
Anti-Hu antibodies should be tested in patients with simultaneous resentation of motor, sensitive and autonomic neuronopathy.

Palavras Chave

Anti-Hu syndrome, ganglionopathy, motor neuron disease

Área

Neuroimunologia