英文病例一则

PRESENTATION of CASEDr. Patricia L. Musolino (Neurology): An 18-year-old man was transferred to this hospital because of blurred vision, dysarthria, and ataxia, which had reportedly progressed to coma.The patient had been well until 6 p.m. the evening before admission, when he had an abrupt onset of weakness, shaking and buckling of his legs, and clumsiness of his right hand. Approximately 20 minutes later, his speech became slurred; his parents transported him to the emergency department at another hospital. While in transit, the dysarthria worsened and the patient reported blurred vision. On arrival at the hospital 1 hour after the onset of symptoms, he required assistance getting into a wheelchair. He reported being off-balance and light-headed, with blurred vision and pain at the base of the right side of the skull. On examination, he was alert and oriented but slow to respond, and he intermittently laughed inappropriately. The vital signs were normal, and the oxygen saturation was 100% while he was breathing ambient air. On neurologic examination, the pupils and extraocular movements were reportedly normal, without nystagmus. The speech was slurred. The first cranial nerve was not tested; results of testing of the other cranial nerves were normal. The deep-tendon reflexes were 1+ and symmetric. Alternating movements of the hands were slow, with some lack of coordination; his performance on finger-to-nose testing was accurate.The white-cell count was 8600 per cubic millimeter, with 80% granulocytes, 14% lymphocytes, and 6% monocytes. The remainder of the complete blood count and the results of renal- and liver-function tests were normal, as were the levels of electrolytes and glucose, measurements of arterial blood gases performed while the patient was breathing ambient air, and other test results. Screening of the blood for alcohol and the urine for toxins and drugs was negative. Computed tomography (CT) of the head was reportedly normal. The patient was admitted to the other hospital. A lumbar puncture was performed; tube 1 of the cerebrospinal fluid (CSF) appeared bloody, with gradual clearing. Reportedly, the white-cell count was 9 per cubic millimeter in tube 1 and 1 per cubic millimeter in tube 4, and the red-cell count was 10,000 per cubic millimeter in tube 1 and 54 per cubic millimeter in tube 4. The protein level was 58 mg per deciliter, and the glucose level 62 mg per deciliter (3.4 mmol per liter). No organisms were seen on Gram's staining.Overnight, the patient frequently flailed his arms and legs, according to his mother. On examination the next morning, he was alert, oriented, communicative, and restless, with intermittent movements of the arms; strength was normal, eye movements were full, pupils were reactive, and there was mild ataxia. At approximately 11:30 a.m., difficulty walking developed, violent shaking of his legs and arms began, and he became unable to speak. Magnetic resonance imaging (MRI) performed 30 minutes later, after the administration of lorazepam (5 mg in 1-mg increments to decrease motion artifact),reportedly revealed abnormal signal changes in the brain stem.The patient was transferred to the intensive care unit (ICU) at the other hospital. On examination, he was somnolent but arousable to painful stimuli. During the next 3 hours, he appeared to see and hear his mother but was unable to speak. Shortly before transfer to this hospital, extensor posturing with clenched fists and rigid arms developed. On arrival of the transport team, succinylcholine, propofol, rocuronium, and fentanyl were administered and the trachea was intubated. The patient was transported by helicopter to this hospital, arriving approximately 23 hours after the onset of symptoms. Lorazepam and additional fentanyl were administered in transit.The patient's history of birth and development was normal, and he had been previously healthy, with a learning disability. He had been scratched by a neighbor's dog 3 weeks before admission; the vaccination status of the dog was unknown, but the animal continued to appear well. Approximately 2 days before admission, the patient had removed an embedded tick, which was not engorged, from his skin. He took no medications and had no allergies. He lived with his family, had attended 3 years of high school, and worked in a service position. His grandfather and mother had had transient ischemic symptoms, a maternal aunt had died of neuronal ceroid lipofuscinosis, and a half-sibling had a learning disability.The patient was admitted to the pediatric ICU at this hospital. On examination, he was mechanically ventilated with an endotracheal tube. There was tonic deviation of the head to the left side. The pupils were 3 mm in diameter and weakly reactive, corneal reflexes were present, and there was no oculocephalic reflex. The tone in the arms was slightly increased, and they were held in the extended position. The fingers were firmly flexed but without Hoffmann's sign (flexion of the terminal phalanx of the thumb and of the second and third phalanges of another finger on tapping the index, middle, or ring finger). The legs were extended and tremulous, with greatly increased extensor tone. There was sustained clonus, and the plantar reflexes were extensor. The patient responded to noxious stimulation with increased extension of the arms and triple flexion (hip, knee, and ankle) of the legs. The remainder of the examination was normal.The white-cell count was 10,900 per cubic millimeter (reference range, 4500 to 13,000), with 85% neutrophils (reference range, 40 to 62), 9% lymphocytes (reference range, 27 to 40), and 6% monocytes (reference range, 6 to 11); the serum level of phosphorus was 0.9 mg per deciliter (0.3 mmol per liter; reference range, 2.6 to 4.5 mg per deciliter [0.8 to 1.5 mmol per liter]); and results of coagulation and renal-function tests were normal, as were blood levels of hemoglobin, platelets,D-dimer, lactic acid, electrolytes, calcium, magnesium, lipase, amylase, total protein, and albumin.Ten minutes after admission, a diagnostic procedure was performed.sp-3.6.0bovidweb.cgi&S=BLIOPDPGMOHFDHNBFNPKADEGKDFDA A00&Link+Set=S.sh.26.27.31.62%7c17%7csl_10。