Clinical, paraclinical aspects and complex therapeutical approaches in a patient with incomplete paraplegia, post thoracic menigioma surgically treated, in neurofibromatosis context

Abstract

Introduction: Neurofibromatosis - type 1 (NF1) and type 2 (NF2) - are genetic disorders of the nervous system that can affect the growth and development of nerve cell tissue and so can determine severe or rather permanent sequels. NF2 implies usually multiple tumors on the cranial and spinal nerves and it is less common than NF1. The most frequent symptom of NF2 is hearing progressive loss, as a consequence of auditory nerves affection and appears at early ages or at twenties. The evolution of a patient with NF2 depends on the number and location of tumors and some of them might develop a life-threatening or disabling condition. With a prompt diagnosis and an appropriate therapy it can be improved the patient prognosis and QOL. Materials and Methods: This paper presents the case of a 43-year-old patient, with personal antecedents of hearing dysfunction, diagnosed in 2013 with neurofibromatosis that was hospitalized at the IV Neurosurgery Clinic of TEHBA in January 2019 and suffered a re-intervention for removal of the spinal cord tumor (psammomatous meningioma) and with spinal cord decompression. In our clinic, the patient was admitted for incomplete AIS/Frankel C paraplegia, he had initially followed a complex nursing program and subsequently a rehabilitation adequate program. The patient was assessed functionally using the following scales: AIS / Frankel, modified Ashworth, Functional Independence Measure (FIM), Life Quality Assessment (QOL), FAC International Scale, Independence Assessment Scale in Daily Activities (ADL / IADL), Walking Scale for Spinal Cord Injury (WISCI). Results: The paraclinical assessments (cerebral and spinal cord MRI) detect multiple cerebral tumors and micro-nodules adjacent to the lumbar spinal roots, which, associated with the bilateral acoustic neurinoma (diagnosed in 2013), contributed to the suspicion of the NF2 diagnosis. The patient had two admissions in our clinic division, benefited from a complex neuro-muscular rehabilitation program, having a favourable evolution, with an increase in the evaluated scales scores, now performing walking with a support from another person in walking frame, as well as sphincter re-education, with the neurogenic bladder remission. Conclusions: Even if there is no cure for neurofibromatosis and no standard treatment, it is important to promptly diagnose such a rare disease and to give an adequate treatment (AINS or other analgesic drugs, surgery, chemotherapy or radiation –when it’s needed, or psychotherapy) for controlling symptoms and also a personalized rehabilitation program (including nursing measures) enhancing including patient's quality of life. Key words: paraplegia, neurofibromatosis, neuro-muscular rehabilitation,