Acute transverse myelitis at the conus medullaris level after rabies vaccination in a patient with Behçet's disease

Abstract

Case report: A 25-year-old man with Behçet's disease was admitted because of weakness of the lower limbs and difficulty in urination. He had received a rabies vaccination 2 months previous because he had been bitten by a dog. Findings: Clinical and laboratory findings supported acute transverse myelitis. A hyperintense lesion and expansion at the level of conus medullaris was detected on spinal magnetic resonance imaging. Conclusion: Although neurologic involvement is one of the main causes of mortality and morbidity in Behçet's disease, the factors that aggravate the involvement of the nervous system are still unclear. Vaccination may have been the factor that had activated autoimmune mechanisms in this case. To our knowledge, involvement of the conus medullaris in Behçet's disease after rabies vaccination has not been reported.

Indications:For prevention of rabies in a patient who was bitten by a dog. Coexisting diseases: Behcet's disease, relapsing aphthous stomatitis and genital ulcerations.

Patients:One 25-year-old male patient.

TypeofStudy:A case report describing acute transverse myelitis at the conus medullaris level after Rabipur vaccination in a patient with Behcet's disease.

AdverseEffects:1 patient developed acute transverse myelitis at the conus medullaris characterized by weakness of lower limbs, urinary retention, severe backache, difficulty with urination and defecation, hypoesthesia below the level of L1, absence of lower extremities vibratory and position senses, absence of deep tendon reflexes in the lower extremities, detrusor atony, and atrophy and fasciculations of the left leg muscles.

AuthorsConclusions:Involvement of the conus medullaris in Behcet's disease after rabies vaccination has not been reported previously in the literature. The possibility of an activating factor (eg, vaccination) other than infection has never been mentioned in Neuro-Behcet syndrome. Understanding the activating factors may be helpful for the prevention of neurological involvement and is important for planning follow up and understanding the prognosis. Further reports and investigations are needed.

FreeText:Tests: cerebrospinal fluid (CSF) leukocyte and protein level; muscle strength using Medical Research Council grade scoring; spine magnetic resonance imaging (MRI); vibratory and position senses; and deep tendon reflexes. The active substance of the rabies vaccine was an inactivated rabies virus.

Results:Two months after the administration of the rabies vaccine, the patient experienced weakness of lower limbs which was preceded by severe backache for 12 hours and urinary retention. The weakness progressively worsened, and he began experiencing difficulty with urination and defecation. Muscle strength score was 2/5 at proximal left lower limb, 1/5 at distal left lower limb, 3/-5 at proximal right lower limb, and 1/5 at distal right lower limb. Atrophy and fasciculations of the left leg muscles were noted. There was hypoesthesia below the level of lumbar 1 (L1), and lower extremities demonstrated absence of vibratory and position senses. Absence of deep tendon reflexes was noted in the lower extremities, and plantar responses were extensor bilaterally. CSF examination revealed 110 leukocytes/mm3 with no microorganisms and elevated protein level (114 mg/dL). Spine MRI showed hyperintense lesion and expansion at the level of the conus medullaris. The patient was treated with methylprednisolone, azathioprine, and physiotherapy. He also required intermittent catheterization for urinary retention. After a year, a complete resolution of symptoms was observed and spinal MRI became normal.

DosageDuration:Dosage not stated; given on the day of the bite and on the 3rd and 7th days thereafter.