| In a 73-year-old patient complete areflexia of the cerebral and peripheral nerves following the rupture of an aneurysm of the basilar artery was diagnosed. During apnea testing the spectral analysis of electroencephalography (EEG) revealed an irreversible shift of peak from 6 to 3 Hz within the low-frequency bands. These findings suggest that apnea testing in patients with primary lesion of the brain stem should be carried out only after an isoelectric EEG. ( info) |
72/245. dialysis Disequilibrium syndrome: brain death following hemodialysis for metabolic acidosis and acute renal failure--a case report. BACKGROUND: dialysis disequilibrium syndrome (DDS) is the clinical phenomenon of acute neurologic symptoms attributed to cerebral edema that occurs during or following intermittent hemodialysis (HD). We describe a case of DDS-induced cerebral edema that resulted in irreversible brain injury and death following acute HD and review the relevant literature of the association of DDS and HD. CASE PRESENTATION: A 22-year-old male with obstructive uropathy presented to hospital with severe sepsis syndrome secondary to pneumonia. Laboratory investigations included a pH of 6.95, PaCO2 10 mmHg, HCO3 2 mmol/L, serum sodium 132 mmol/L, serum osmolality 330 mosmol/kg, and urea 130 mg/dL (46.7 mmol/L). diagnostic imaging demonstrated multifocal pneumonia, bilateral hydronephrosis and bladder wall thickening. During HD the patient became progressively obtunded. Repeat laboratory investigations showed pH 7.36, HCO3 19 mmol/L, potassium 1.8 mmol/L, and urea 38.4 mg/dL (13.7 mmol/L) (urea-reduction-ratio 71%). Following HD, spontaneous movements were absent with no pupillary or brainstem reflexes. head CT-scan showed diffuse cerebral edema with effacement of basal cisterns and generalized loss of gray-white differentiation. brain death was declared. CONCLUSIONS: Death is a rare consequence of DDS in adults following HD. Several features may have predisposed this patient to DDS including: central nervous system adaptations from chronic kidney disease with efficient serum urea removal and correction of serum hyperosmolality; severe cerebral intracellular acidosis; relative hypercapnea; and post-HD hemodynamic instability with compounded cerebral ischemia. ( info) |
73/245. Serial S100B levels before, during and after cerebral herniation. Protein S100B has been shown to increase in serum and cerebrospinal fluid (CSF) in various neurological diseases. However, the levels of S100B in conjunction with cerebral herniation have not been studied and the significance of extracerebral S100B has become an important issue. We report on a multi-trauma patient in whom cerebral herniation occurred 2 days after admission. Following this, organ-harvesting procedures were performed for transplantation. We measured serial serum S100B during both the ongoing herniation and the following extracerebral surgery. We found that S100B levels seemed to peak immediately prior to cerebral herniation and then decreased shortly thereafter and concluded that the source of the measured serum S100B in this patient was of predominately cerebral origin. In conjunction with the organ harvesting procedure S100B levels increased, indicating that extracerebral sources of the protein also exist. ( info) |
74/245. Preserved spinal dorsal horn potentials in a brain-dead patient with Lazarus' sign. Case report. The case of a brain-dead patient with complex movements of the extremities (Lazarus' sign) is reported. This is the first description in the literature of short-latency somatosensory evoked potentials (SSEP's) following median-nerve stimulation by a noncephalic reference method. The scalp P14 wave (a far-field positivity with a peak latency around 14 msec that originates from the cervicomedullary junction) disappeared, and the spinal N13 wave (a near-field negativity with a 13-msec peak recorded on the posterior neck and generated by the cervical dorsal horn) was preserved. Respiratory-like movement was also seen in this case. The SSEP. findings support the hypothesis that both Lazarus' sign and respiratory-like movement have a spinal origin. ( info) |
75/245. Difficulties in assessing brain death in a case of benzodiazepine poisoning with persistent cerebral blood flow. Assessing brain death may sometimes be difficult, with isoelectric EEG following psychotrope overdoses or normal cerebral blood flow (CBF) persisting despite brain death in the case of ventricular drainage or craniotomy. A 42-year-old man, resuscitated after cardiac arrest following a suicidal ingestion of ethanol, bromazepam and zopiclone, was admitted in deep coma. On day 4, his brainstem reflexes and EEG activity disappeared. On day 5, his serum bromazepam concentration was 817 ng/ml (therapeutic: 80-150). The patient was unresponsive to 1 mg of flumazenil. MRI showed diffuse cerebral swelling. CBF assessed by angiography and Doppler remained normal and EEG isoelectric until he died on day 8 with multiorgan failure. There was a discrepancy between the clinically and EEG-assessed brain death, and CBF persistence. We hypothesized that brain death, resulting from diffuse anoxic injury, may lead, in the absence of major intracranial hypertension, to angiographic misdiagnoses. Therefore, EEG remains useful to assess diagnosis in such unusual cases. ( info) |
| tissue distribution of lidocaine that was used for endotracheal intubation during cardiopulmonary resuscitation (CPR) was measured in 3 patients who were brain-dead or near brain death. Case 1 was a 69-year-old female whose heartbeat was restored by CPR but stopped 10 hours later. The lidocaine ratios of cerebrum to blood (2.04) and diencephalon to blood (1.01) were within ranges of those found in non-brain-dead patients. Case 2 was a 77-year-old female whose heart resumed beating after CPR but stopped 66 hours later. The lidocaine ratios of cerebrum to blood (5.69), diencephalon to blood (18.7), and cerebellum to blood (11.3) were much higher than those in non-brain-dead patients. Case 3 was a 48-year-old male who had cardiopulmonary arrest following an acute subarachnoid hemorrhage. His heart resumed beating after resuscitation but ceased beating 114 hours after admission. lidocaine was detected only from the cerebrum, cerebellum, and blood clots in the superior sagittal sinus at levels of 0.028, 0.024, and 0.007 mug/g, respectively. tissue distribution of intubation-related lidocaine in brain-dead patients is useful as supplementary data for reviewing hemodynamic changes in their brains during medical treatment. ( info) |
77/245. Biochemical analysis of the cerebrospinal fluid: evidence for catastrophic energy failure and oxidative damage preceding brain death in severe head injury: a case report. OBJECTIVES: To compare biochemical and clinical parameters in a case of fatal severe traumatic brain injury (TBI) with secondary insult. DESIGN AND methods: A TBI patient was catheterized for intracranial pressure (ICP) monitoring and cerebrospinal fluid (CSF) analysis of ascorbate, malondialdehyde, oxypurines, and nucleosides. RESULTS: Oxidative brain damage preceded ATP catabolite increment in the CSF even with ICP below 20 mm Hg. Sustained oxidative stress caused irreversible energy state derangement followed by a refractory ICP rise. Massive oxypurine and nucleoside release was recorded 36 h before brain death. CONCLUSIONS: Molecular events, detected by biochemical CSF analysis and preceding modification of clinical parameters in severe TBI with secondary insult, are discussed. ( info) |
| The first case of domino liver transplantation from a brain-dead donor in japan is described. A 49-year-old man with familial amyloidotic polyneuropathy received a cadaver liver, and his native liver was transplanted into a 53-year-old man with polycystic liver and kidney disease. The cadaveric liver allograft was transplanted by the conventional technique. The graft taken from the first recipient had four outflow orifices (the left, middle, and right hepatic veins, and upper vena cava), for which a single orifice was created at the back table. This graft was transplanted in piggy-back fashion. The first recipient developed acute rejection on day 13 and hepatic artery stenosis on day 36. These were treated by steroid recycle therapy and percutaneous transarterial angioplasty. He was discharged on day 57 with normal liver function. The second recipient underwent re-operation for bleeding from the right adrenal gland and left thoracic cavity. He was diagnosed with acute rejection on day 7, which was treated by steroid pulse therapy. He was discharged uneventfully on day 39 with normal liver function. ( info) |
79/245. diffusion MRI in the postmortem brain: case report. Postmortem brain of a ten-month-old child was examined by MR imaging, and diffusion MR imaging at the 12th hour after death in order to disclose the cause of death. There were basal ganglion lesions indicating a mitochondrial disorder. There was a prominent difference between the ADC values of the white matter (0.28 /-0.04 x 10(-3) mm2/s) and cortex (0.42 /-0.04 x 10(-3) mm2/s), and this was statistically significant (p< 0.0001). This difference suggested that in the postmortem brain the conditions in the white matter leading to restriction of movement of water molecules are more severe than that in the cortex. ( info) |
80/245. Late onset N-acetylglutamate synthase deficiency caused by hypomorphic alleles. N-acetylglutamate (NAG) is a unique cofactor that is essential for the conversion of ammonia to urea in the liver. N-acetylglutamate synthase (NAGS) catalyzes the formation of NAG. Deficiency of NAGS causes a block in ureagenesis resulting in hyperammonemia. Although a number of mutations have been identified in the NAGS gene, their effects on NAGS enzymatic activity have not been examined. We describe here three mutations in two families with NAGS deficiency. Studies of the purified recombinant mutant proteins revealed deleterious effects on NAGS affinity for substrates, and on the rate of catalysis. These studies provide a better understanding of the function of NAGS, and the mechanisms for deleterious effect of mutations causing inherited NAGS deficiency. ( info) |