Faculty Bibliography

Report of Case: We report a healthy teenager with involuntary nocturnal tongue biting resulting in recurrent tongue injury. Causes for tongue biting during sleep in children include seizures, bruxism, faciomandibular myoclonia, hypnic myoclonia, and rarely geniospasm, which has been described as a rare inherited movement disorder accompanied with chin quivering. In the absence of family history, we diagnosed our patient with sporadic geniospasm based on polysomnographic findings with good response to clonazepam. Geniospasm should be considered in the differential diagnosis of cases with unexplained tongue injury in sleep.

INTRODUCTION: We present a case of disseminated MSSA infection causing devastating ischemic sequelae of septic emboli. CASE PRESENTATION: The patient is a 66 year-old man presenting to the ER after he fell off his couch and could not get up. He had chronic back pain and weakness and swelling of both legs. His medical history included recent dental work, acupuncture for shoulder pain, and ORIF for bilateral wrist fractures. On presentation, he was febrile, tachycardic, tachypneic, and hypoxemic, with 4/5 muscle strength throughout. Chest X-ray showed lower lobe infiltrates. Urinalysis suggested infection. He was given broad-spectrum antibiotics and IV fluids. He was transferred to the medical ICU for hypotension. His weakness rapidly progressed to quadriplegia, with shallow, labored breathing. He was intubated for hypoxemic respiratory failure and septic shock requiring vasopressors. CT imaging of the head, cervical and thoracic spine was negative. Lumbar puncture retrieved purulent CSF with a neutrophilic pleocytosis and gram-positive cocci. Dexamethasone was added for bacterial meningitis. A cervical spine MRI showed C3-C7 cord signal abnormality of the central gray matter, concerning for spinal cord infarction. MSSA grew from blood, urine and CSF cultures. Antibiotics were narrowed to nafcillin. A chest CT showed peripheral septic emboli in the lungs. A transthoracic echocardiogram showed large, shaggy aortic and tricuspid valve vegetations. The patient developed massive upper GI bleeding. Endoscopy revealed diffuse ischemic ulcerations, requiring radiologically-guided embolization for hemostasis. His mental status remained depressed; he intermittently answered questions by blinking. He did not regain any neurologic function. He developed progressive multiorgan system failure. After discussion with his family, in keeping with his known wishes in the case of a terminal condition, the patient was palliatively extubated and expired soon thereafter. DISCUSSION: Our patient represents an uncommon case of widely disseminated MSSA infection due to infective endocarditis of the tricuspid and aortic valves. The sequelae included a descending UTI, septic emboli to the lungs, and septic emboli causing ischemic ulceration of the duodenal mucosa, vertebral artery occlusion, and cervical spinal cord infarction resulting in quadriplegia. The patient's MSSA infection was likely due to bacteremia resulting from acupuncture treatments for shoulder pain. He was hyperglycemic on admission, his HbA1c was 6.7%. Impaired glucose tolerance or uncontrolled undiagnosed diabetes mellitus likely predisposed the patient to dissemination of MSSA infection. CONCLUSIONS: This case illustrates rapidly progressive MSSA infective endocarditis, with ischemic injury from septic emboli contributing to multiorgan system failure and devastating neurologic injury

STUDY OBJECTIVES: To examine the impact of gender in narcoleptic patients on timeliness of diagnosis, symptomology, and health and lifestyle impairment. METHODS: This is a cross-sectional study of 109 consecutive patients (68 women) with newly diagnosed narcolepsy with and without cataplexy, from a University sleep disorders center. Consecutive patients were administered an 8-page questionnaire at the time of their diagnosis regarding sleep habits, medications, and medical conditions, lifestyle impairments, as well as details regarding narcolepsy-related symptoms. RESULTS: Men and women presented with remarkably similar narcolepsy related symptoms, yet women were more likely to be delayed in diagnosis; 85% of men were likely to be diagnosed by 16 years after symptom onset, compared to 28 years in women. More women were likely to remain undiagnosed at any given time point after symptom onset (hazard ratio for diagnosis of men compared to women 1.53; 95% CI 1.01-2.32; p = 0.04). Men and women reported similar degree of subjective sleepiness as measured by the Epworth Sleepiness Scale (mean 16.2 +/- 4.5; p = 0.18), though women demonstrated significantly more severe objective sleepiness on multiple sleep latency testing (MSLT) (mean sleep latency in women = 5.4 min (+/- 4.1), in men 7.4 min (+/- 3.5); p = 0.03). Despite being more objectively sleepy, women were less likely to report lifestyle impairments in the areas of personal relationships (71% men, 44% women, p = 0.01) and physical activity (36% men, 16% women, p = 0.02), but were also more likely to self-medicate with caffeine (63.4% men, 82.4% women; p = 0.03). CONCLUSIONS: Narcolepsy impacts men and women's health and lifestyle differently, and may cause delays diagnosis for women.