Progressive Weakness and Dyspnea in a 14-Year-Old Boy

Danielle D. DeCourcey, MD; Mark Wainwright, MD, PhD; Jason M. Kane, MD


April 02, 2018

Physical Examination and Work-up

The physical examination reveals an afebrile, ill-appearing teenager, with a heart rate of 118 beats/min, a respiratory rate of 28 breaths/min, a blood pressure of 168/122 mm Hg, and an oxygen saturation of 93% while breathing room air. Auscultation of the lungs reveals diffuse, poor aeration. His heart sounds are normal, without any appreciable murmur. His strength is symmetric but diminished to 2/5 in his lower extremities and 4/5 in his upper extremities (5/5 being normal strength). The patient's sensation is intact to light touch, but he has a loss of vibratory sense. He has no deep tendon reflexes in his lower extremities, diminished deep tendon reflexes (1+) in his upper extremities, and absent plantar reflexes. Cranial nerves II-XII are intact; however, he has a weak cough and gag reflex, with impaired handling of secretions. The remainder of his examination is unremarkable.

The patient is intubated for progressive respiratory distress and loss of airway-protective reflexes. He is fluid-resuscitated with a liter of intravenous normal saline. An electrocardiogram (ECG) is obtained, which demonstrates sinus tachycardia. The initial laboratory analysis, including a complete blood cell (CBC) count and a basic metabolic panel, is within normal limits. A lumbar puncture is performed, with an opening pressure of 15 cm H20. The cell count and Gram stain of the cerebrospinal fluid (CSF) demonstrates 2 white blood cells per high power field, 4 red blood cells per high power field, and no organisms. Additional analysis of the CSF shows a protein concentration of 96 mg/dL (960 mg/L) and glucose concentration of 72 mg/dL (3.99 mmol/L). The patient is sent for MRI of his brain and spine (see Figure 1) and is transported to the pediatric intensive care unit (ICU) for further management.

Figure 1.


Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.