Spinal thoracic tuberculoma in neurofibromatosis type-1

The clinical manifestations of neurofibromatosis type-1 have in common the presence of neurofibromas, schwannomas and cafe`-au-lait macules, which can potentially appear within any organ system of the body, involving primarily the skeleton, skin and soft tissues. The spinal thoracic tuberculoma in neurofibromatosis is exceptional. We report here, the case of a 31-year-old male with neurofibromatosis type-1 who presented a year agoa spinal thoracic pain. The evolution was marked by the appearance of a dorsal para-vertebral mass, progressively increasing in volume for 03 months. Neurological Examination revealed normal muscle bulk and tone, and power was grade 5/5. Deep tendon reflexes were brisk and the Babinski sign was present. The sensory deficit was present below the D9 level. The remainder of the examination found a voluminous dorsal para vertebral mass ovoid, renitent, and adhering to the superficial and the deep planes. There were also "café-au-lait" spots disseminated on his body. The imagery showed a formation with respect to D11-D12, isointense on T1 and T2, heterogeneously enhanced after contrast with central necrosis, with a foraminal starting point measuring 85x78x66 mm, with external canal extension. Laminectomy D11-D12 was performed with incomplete excision of the lesion. The histological examination showed casein-follicular vertebral tuberculosis, with secondary abdominal changes. Then the patient was put on anti-bacillary drugs. The evolution was marked two weeks later by the reappearance of the back pain and the back mass. On the 42nd day of treatment, the patient had a febrile consciousness disorder with a GCS of 8, right mydriasis and meningeal stiffness. Paraclinic investigations revealed tuberculosis meningoencephalitis, responsible for an active ventricular hydrocephalus associated with a left temporoparietal extrudal hematoma. An external ventricular shunt was performed as well as a left temporo-parietal extrudal hematoma evacuation. The patient died 7 days later in a poly visceral failure chart.