Paraplegia in a Teenage Girl

Abstract

CASE A 16-year-old female presented to the Emergency Department unable to walk. She reported having fallen on the stairs and sustaining a right knee injury 3 months prior. Since that time, she had increasing difficulty walking to the point that her family had obtained a wheelchair for her. While the pain from the injury subsided, her left leg became weaker, which the family attributed to the left leg having “taken the strain” due to the right-sided injury. Over the past week, she developed difficulty with urination and reduced perianal sensation on wiping. She denied back pain or pain elsewhere. She otherwise felt well with no fevers and good appetite. On review of systems, no other symptoms were reported. Her only notable medical history was severe obesity, with a weight of 108 kg. She was born in the United Kingdom to parents who originated from the Democratic Republic of Congo. She had no travel history outside of the United Kingdom. She had no known contact with infectious persons. She had been fully immunized according to the UK vaccination schedule. She lived on the third floor of an apartment block with her parents and siblings. On examination, she appeared well. She was orientated to time, place and person and was lucid in conversation. She had a temperature of 36.7°C, heart rate of 99 beats/min, respiratory rate of 22 breaths/min, blood pressure of 133/85 mm Hg and oxygen saturation of 99% in ambient air. Cardiovascular, respiratory and abdominal examinations were unremarkable. There was no jaundice, anemia, clubbing, cyanosis, edema or lymphadenopathy noted. However, she had no detectable strength in the muscle groups throughout both lower limbs and had increased tone and absent reflexes. There was a sensory level at T10, perianal anesthesia and reduced anal tone. Upper limb neurologic examination was normal. Her back was nontender and had no external signs of trauma. Laboratory studies revealed a hemoglobin of 122 g/L; white cell count of 5.69 × 109/L with differential of 43.7% neutrophils, 40.1% lymphocytes, 9.3% monocytes, 6.7% eosinophils and 0.2% basophils; and platelet count 270 × 109/L. Renal, liver and thyroid function studies were normal. She had a prothrombin time 10.3 seconds, activated partial thromboplastin time of 23 seconds, and fibrinogen of 4.90 g/L. C-reactive protein was 10.6 mg/L, and erythrocyte sedimentation rate was 24 mm/h. Angiotensin-converting enzyme was 53 U/L. Serologic testing for hepatitis B, hepatitis C, HIV and human T-lymphotropic virus were all negative, but serum IgG for varicella-zoster virus was positive. She underwent emergency magnetic resonance imaging (MRI) of the spinal cord, which demonstrated a well-circumscribed, lobulated and enhancing intramedullary mass lesion extending from the inferior aspect of T10 to the inferior aspect of T11, almost filling the vertebral canal at this level. There was associated edematous change within the spinal cord between T7 and the conus at L1. The vertebrae and vertebral disks were normal. Dexamethasone was commenced, and she was transferred to the regional neurosciences specialty center. Cerebrospinal fluid was clear and colorless, with <1 white cell per mm3, and microscopy was negative for organisms including acid-fast bacilli. CSF was also negative by polymerase chain reaction for Varicella-Zoster virus, Herpes-Zoster virus1 and Herpes-Zoster virus2, Epstein-Barr virus, cytomeganovirus, enterovirus and Mycobacterium tuberculosis complex. CSF had total IgG of 51 mg/L with albumin 231 mg/L. Oligoclonal bands were negative in both serum and CSF. Serum testing for aquaporin 4, antineuronal, antinuclear, antineutrophil cytoplasm, double-stranded DNA and extractable nuclear antigen antibodies were all negative. She underwent further MRI of the brain and spinal cord (Fig. 1), which demonstrated avid, homogeneous enhancement of the intramedullary lesion and an additional left cerebellar hemispheric lesion without any meningeal enhancement. The lesions were isointense on T1 and hypointense on weighted T2 sequences, without evidence of restricted diffusion. Subsequent biopsy of the spinal lesion led to the diagnosis.FIGURE 1.: MRI images at presentation. A: Gadolinium-enhanced T2-weighted sagittal view of spine demonstrating intramedullary enhancement; (B) coronal fluid-attenuated inversion recovery sequence of brain demonstrating left cerebellar lesion without associated enhancement.