A 25-year-old woman presented to the emergency department (ED) with a history of 2 seizures before presentation. According to the family, the first seizure occurred around 3 PM on the day of presentation. It was described by the paramedics and bystanders as a generalized tonic-clonic event lasting about 3 minutes. The patient had a second seizure en route to the hospital and another in the ED. She exhibited confusion between episodes. The third episode occurred about 3:30 PM and was witnessed by ED physicians who described generalized body stiffening followed by left upper extremity elevation, left lower extremity straightening, rightward head turn, urinary incontinence, and an oxygen desaturation to 67% on pulse oximetry. Subsequently, the patient was confused and unable to detail her medical history. Her parents provided her history, which was notable for acute myelogenous leukemia (AML) diagnosed at 18 months of age and treated with radiation and chemotherapy (systemic and intrathecal). The patient had had a basal cell carcinoma surgically excised from the right posterior portion of her scalp in 2006. She was taking no medications, and the family history was negative for seizures, endocrine disorders, or neoplasms. She had no history of tobacco or illicit drug use, falls, or head trauma. Her parents reported no preceding fever, infectious symptoms, or other systemic complaints.Physical examination revealed the following: temperature, 36.4ºC; blood pressure, 108/61 mm Hg; heart rate, 112 beats/min (sinus tachycardia); respiratory rate, 21 breaths/ min; and oxygen saturation, 100% on 3 L/min of oxygen via nasal cannula. The patient was young and well-nourished but unable to cooperate or follow commands. Head and neck examination revealed a supple neck and no evidence of tongue lacerations or oropharyngeal lesions. Hair loss and a scar from previous excision of the basal cell carcinoma were evident on the posterior aspect of the scalp. Lung sounds were notable for rhonchi bilaterally, which were transmitted from the oral airway. Cardiovascular examination revealed tachycardia but no appreciable murmurs, rubs, or gallops. Abdominal examination findings were unremarkable. Examination of the extremities revealed no cyanosis, edema, or skin lesions. Neurologically, the patient was unresponsive verbally and did not obey commands. Her eyes were closed and did not open to noxious stimuli. There was no evidence of facial asymmetry. Cranial nerve examination revealed 6-mm pupils that were briskly reactive to light bilaterally. Funduscopic examination yielded normal findings and no papilledema. The remainder of the patient's brainstem reflexes, including cough and gag, were intact. Segmental motor strength could not be assessed. However, noxious stimuli elicited purposeful localizing and withdrawal responses in upper and lower extremities symmetrically. Deep tendon reflexes were 2+ (Medical Research Council scale) and symmetric in the biceps, triceps, patella, and Achilles tendon. The left toe was upturning (Babinski sign...
A 79-year-old woman with a history of prior right frontal infarction presented with sudden inability to speak (figure, A). Examination revealed anarthria, yet intact comprehension, writing, and limb function (video). Voluntary tongue, swallowing, and mouth movements were absent, while cough and swallow reflexes were preserved (video). An acute left hemispheric ischemic stroke causing Foix-ChavanyMarie syndrome (FCMS) was diagnosed, and IV recombinant tissue plasminogen activator was administered. Left frontal infarction was confirmed on MRI ( figure, B).FCMS involves lesions of the bilateral frontal operculum 1 or the cortical-subcortical areas of primary motor cortex. 2 Voluntary control of facio-pharyngo-glosso-masticatory muscles is lost, while reflex movements and limb strength are preserved.
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