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Título

A RARE CASE OF PARANEOPLASTIC LONGITUDINALLY EXTENSIVE MYELOPATHY, SECONDARY TO MALIGNANT PHEOCHROMOCYTOMA

RESUMO

Male patient, 60 years old, with hemophilia type A diagnosed at the age of 13 years old and HCV diagnosed in 1983, with cure control in 2012. 50 days ago from admission, he noticed painless inguinal lymph nodes. After 15 days, he began dysesthesia in the cervical region and upper limbs. He evolved with severe neck pain, dorsal irradiation, worsening of movement and motor symptoms, associated with nausea, vomiting and hiccups. 10 days before admission, he started sensory-motor symptoms in the lower limbs predominant asymmetric to the left and, after 4 days,presented constipation and urinary incontinence. After 5 days, he presented restriction on ambulation and loss of muscle strength in the lower limbs and was hospitalized. On admission neurological examination showed grade V strength in the upper limbs, grade III in right lower limb,grade II in left lower limb (preserved toe dorsiflexion, grade IV plantar flexion of the toes).Babinski sign present bilaterally. Laboratory tests showed: LDH 795; Anti-HVC 166. Normal rheumatological tests. CSF: colorless, cell 1, proteins 119 mg/dl; missing oligoclonal bands, anti-Aquaporin 4 negative. Total abdominal CT: Retroperitoneal and pelvic lymph node enlargement.Nodule in the body of the left adrenal gland. Neuroaxis MRI: Capture annular lesion, encapsulated with perilesional edema in the left frontotemporal region. Cervical spinal cord injury with irregular contrast uptake, bulb-sacral enhancement. Biopsy left inguinal lymph node tissue with immunohistochemistry: malignant, metastatic pheochromocytoma. During hospitalization, he evolved with unprecedented quadriparesis and seizures, being submitted to orotracheal intubation and transferred to the ICU. It was decided to perform pulse therapy with methylprednisolone 1g/5 days, with no clinical response.Discussion: Paraneoplastic longitudinally extensive myelopathy is a rare event, more associated with neoplasms of bones, liver, lymph nodes and lung. Literary evidence of malignant pheochromocytoma with intramedullary involvement is scarce and constitutes a diagnostic and therapeutic challenge. In this case, in addition to extensive spinal cord involvement, brain involvement is evident.

Final Conclusions: The case in question is notable for the rarity of the clinical presentation. The unfavorable evolution is due to the extent of the lesion and concomitant brain involvement, with no previous reports of association with malignant pheochromocytoma in the literature.

Palavras Chave

PARANEOPLASTIC LONGITUDINALLY EXTENSIVE MYELOPATHY ; MALIGNANT PHEOCHROMOCYTOMA