CASE1-Seizures and Flaccid Quadriparesis in a 16-Year-Old Boy
نویسنده خبر : مدیر سایت    
تاریخ درج خبر : 1397/5/9

A 16-year-old boy presents to the emergency department with a 1-week history of weakness in

both lower extremities, which progressed to involve both arms over the course of 1 day. He also complains of dysesthesia. No sphincteric dysfunction is noted.

Two weeks before presentation, he had been treated for an upper respiratory tract infection. He has no history of diarrhea, vomiting, or abdominal pain. He is a nonsmoker and is not taking any medications on regular basis. His family history is unremarkable.

Physical Examination and Workup

His vital signs include an oral temperature of 98.6°F, regular pulse of 70 beats/min, blood pressure of 120/70 mm Hg. His Glasgow Coma Scale score is 15/15, and he is completely alert and oriented to person, place, and time. He has flaccid quadriparesis with hyporeflexia and bilateral mute planter response. Sensations are intact.

Cranial nerve examination findings are unremarkable. Signs of meningeal irritation are absent. His abdomen is soft and nontender. No clinical evidence suggests organomegaly or ascites. His bowel sounds are audible and normal. The patient’s precordial examination reveals normal heart sounds without murmurs. His lungs are clear upon auscultation, with a normal respiratory rate and effort.

Laboratory analysis reveals a normal complete blood cell count and erythrocyte sedimentation rate. Aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and creatinine concentrations are normal; electrocardiography, anteroposterior chest radiography, and abdominal ultrasonography findings also are unremarkable. Nerve conduction studies show abnormalities consistent with demyelination. MRI of the cervical spine with contrast is normal.

On day 2 of admission, the patient becomes drowsy and subsequently has multiple generalized tonic-clonic seizures. His Glasgow Coma Scale score drops to 5/15, and he is moved to the intensive care unit for endotracheal intubation and assisted ventilation. He is started on empiric acyclovir and ceftriaxone along with sodium valproate, and his workup is further extended.

MRI of the brain (Figures 1-4) reveals bilateral, asymmetric areas of high signal intensity in the subcortical and deep white matter on T2-weighted/fluid-attenuated inversion recovery (FLAIR) sequences.





Figure 4.

Lumbar puncture for cerebrospinal fluid (CSF) analysis shows an elevated CSF protein of 0.77g/L, a lymphocytic pleocytosis (280 white blood cells/cmm, with 95% lymphocytes), and a normal glucose concentration. Polymerase chain reaction of CSF for herpes simplex virus is negative. Electroencephalography findings are consistent with diffuse encephalopathy (Figure 5).




Based on the history, physical examination, and workup, which of the following is the likely diagnosis?


  1. Viral encephalitis with Guillain-Barré syndrome
  2. Bickerstaff encephalitis
  3. Acute disseminated encephalomyelitis
  4. Spinal cord compression




Which of the following treatments is the best option for the patient described in this case?

  1. Intravenous immunoglobulin
  2. Plasmapheresis
  3. High-dose intravenous corticosteroids
  4. Intravenous immunoglobulin + corticosteroids
  5. Intravenous acyclovir




Which of the following is the most likely neuropathogenesis of ADEM?

  1. Central nervous system infection
  2. Malignancy
  3. Vasculitis
  4. Demyelination
  5. Paraneoplastic syndrome




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