A 43-year-old man presented to our medical centre with bilateral lower extremity weakness, severe burning electric such as sensation in both lower extremities, and erectile dysfunction that was preceded by lower back pain. He is sexually active and overweight with no significant past medical history except for a treated sexually transmitted gonorrhoea infection. He has no family history of relevant neurologic disease.
His symptoms progressed over 1 month with worsening neuropathic pain in both lower extremities, paraesthesia starting at the umbilicus and radiating down to the medial thighs, inability to walk long distances due to lower extremities weakness and claudication, erectile dysfunction, dribbling and slow urine stream, and constipation. He underwent extensive workup that showed longitudinal transverse myelitis. Cerebrospinal fluid (CSF) studies showed mildly elevated protein (0.56 g/L), normal sugar (55 mg/dl), absent pleocytosis, and no oligoclonal bands. Magnetic resonance imaging (MRI) of the brain and cervical spine was negative, while MRI of the dorsal spine showed a high T2 signal extending from T4 to T9 vertebrae with enhancement at the T7-T8 level.
He received intravenous pulse steroids twice with no improvement, followed by a tapering dose of oral steroids without any improvement or stabilization of symptoms; so, he presented for further work up.
Examination
His physical exam was notable for a sensory level at T10 with allodynia in both lower extremities, very mild weakness in hip flexion on the right (4+/5), and an antalgic gait.
MRI of the dorsal spine was repeated at our institution showing the same longitudinally extensive dorsal lesion with diffuse cord oedema and enhancement. The MRI also showed thickened epidural fat tissue encircling and effacing the CSF at these levels. This was followed by a computed tomography (CT) myelography that showed near complete obstruction of CSF flow extending from T4 to T9 vertebrae. Autoimmune panel including antinuclear antibody, anti-Sjögren's syndrome-related antigen A autoantibodies, anti-Sjögren's syndrome-related antigen B autoantibodies, and antineutrophil cytoplasmic antibodies was negative. Infectious workup, including Brucella titres, human T-lymphotropic virus serology, Toxocara antibodies, human immunodeficiency virus, and syphilis serology, was also negative. Anti-aquaporin antibodies and anti-myelin oligodendrocyte glycoprotein antibodies were negative too. Angiotensin converter enzyme, cobalamin, copper, and calcium levels were normal in blood.
With these MRI findings and negative autoimmune, infectious, demyelinating, and metabolic workup neurosurgical evaluation recommended T4–T10 laminectomy for decompression and epidural fat removal. After surgery, the patient reported a precipitous and significant improvement of his back pain, medial thighs burning sensation, and lower extremity weakness and claudication but persistent paraesthesia, neuropathic pain, and erectile dysfunction.
Four months later, during follow-up in the clinic, the patient reported persistence of his erectile dysfunction, with some remnant neuropathic pain, with normal motor function. A follow up MRI and a CT chest were performed. This was followed by bronchoscopy and mediastinal lymph node biopsy that showed noncaseating granulomas.
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