Management of severe cerebral edema in the metabolic encephalopathy of Reye-Johnson syndrome

1978 ◽  
Vol 48 (6) ◽  
pp. 903-915 ◽  
Author(s):  
Joan L. Venes ◽  
Bennett A. Shaywitz ◽  
Dennis D. Spencer

✓ Fifteen critically ill children with the diagnosis of Reye-Johnson syndrome were treated with techniques developed to maintain adequate cerebral perfusion pressure and levels of circulating blood glucose. One child died, three sustained neurological deficit, and nine children (70%) recovered without significant neurological dysfunction. The techniques developed during the period these children were treated, the indications for their use, and factors that can interfere with maintaining adequate cerebral perfusion in patients with increased intracranial pressure from metabolic encephalopathy are described. The results suggest that neurological damage in this syndrome results from neuronal injury secondary to inadequate cerebral perfusion and/or hypoglycemia, and that neurological dysfunction like hepatic dysfunction should produce minimal mortality and morbidity if cerebral perfusion and adequate levels of circulating blood glucose are sustained during the period of increased intracranial pressure and liver failure.

1978 ◽  
Vol 48 (3) ◽  
pp. 329-331 ◽  
Author(s):  
James E. Cottrell ◽  
Katie Patel ◽  
Herman Turndorf ◽  
Joseph Ransohoff

✓ Because of the ability of sodium nitroprusside (SNP) to dilate cerebral blood vessels, intracranial pressure (ICP) should increase with its use. In patients with vascular intracranial tumors following SNP (0.01%) infusion, ICP increased from 14.58 ± 1.85 to 27.61 ± 3.33 torr (p > 0.0005) and cerebral perfusion pressure decreased from 89.32 ± 3.5 to 43.23 ± 4.60 torr (p < 0.0005) when the mean arterial pressure had reduced by 33%. These results suggest that SNP not be used in patients with raised ICP unless previous measures have been taken to improve intracranial compliance.


1993 ◽  
Vol 78 (2) ◽  
pp. 297-300 ◽  
Author(s):  
Zain Alabedeen B. Jamjoom ◽  
Vinita Raina ◽  
Abdulfattah Al-Jamali ◽  
Abdulhakim B. Jamjoom ◽  
Basim Yacub ◽  
...  

✓ The authors describe a 37-year-old man with the classic clinical features of Hand-Schüller-Christian disease. He presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus secondary to a huge xanthogranuloma involving falx cerebri and tentorium cerebelli. Immunohistochemical and ultrastructural studies failed to demonstrate Langerhans histiocytes, however. The implication of this finding is discussed in light of the recent relevant literature.


2001 ◽  
Vol 95 (4) ◽  
pp. 569-572 ◽  
Author(s):  
Bon H. Verweij ◽  
J. Paul Muizelaar ◽  
Federico C. Vinas

Object. The poor prognosis for traumatic acute subdural hematoma (ASDH) might be due to underlying primary brain damage, ischemia, or both. Ischemia in ASDH is likely caused by increased intracranial pressure (ICP) leading to decreased cerebral perfusion pressure (CPP), but the degree to which these phenomena occur is unknown. The authors report data obtained before and during removal of ASDH in five cases. Methods. Five patients who underwent emergency evacuation of ASDH were monitored. In all patients, without delaying treatment, a separate surgical team (including the senior author) placed an ICP monitor and a jugular bulb catheter, and in two patients a laser Doppler probe was placed. The ICP prior to removing the bone flap in the five patients was 85, 85, 50, 59, and greater than 40 mm Hg, resulting in CPPs of 25, 3, 25, 56, and less than 50 mm Hg, respectively. Removing the bone flap as well as opening the dura and removing the blood clot produced a significant decrease in ICP and an increase in CPP. Jugular venous oxygen saturation (SjvO2) increased in four patients and decreased in the other during removal of the hematoma. Laser Doppler flow also increased, to 217% and 211% compared with preevacuation flow. Conclusions. Intracranial pressure is higher than previously suspected and CPP is very low in patients with ASDH. Removal of the bone flap yielded a significant reduction in ICP, which was further decreased by opening the dura and evacuating the hematoma. The SjvO2 as well as laser Doppler flow increased in all patients but one immediately after removal of the hematoma.


1971 ◽  
Vol 34 (3) ◽  
pp. 405-407 ◽  
Author(s):  
Salvador Gonzalez-Cornejo

✓ The author reports the safe and satisfactory use of Conray ventriculography in 26 patients with increased intracranial pressure and discusses his technique for this procedure.


1982 ◽  
Vol 56 (5) ◽  
pp. 660-665 ◽  
Author(s):  
David D. Weaver ◽  
H. Richard Winn ◽  
John A. Jane

✓ Four patients with unilateral mass lesions are presented in whom bilateral supratentorial subarachnoid pressures were continuously recorded. A significant pressure differential between the ipsi- and contralateral side was documented in each case. The possible relationship of this phenomenon to various factors involved in producing increased intracranial pressure, including cerebrospinal fluid flow dynamics, vascular reactivity, elasticity, and brain tissue pressure, are discussed. This study suggests that supratentorial subarachnoid pressure should be measured ipsilateral to the site of a focal mass lesion.


2021 ◽  
pp. 197140092110193
Author(s):  
Thierry AGM Huisman ◽  
Misun Hwang ◽  
Gunes Orman

A retrospective study was performed evaluating the volume and T2/T1 signal intensity of orbital fat in five children diagnosed with severe global brain swelling and confirmed cessation of cerebral perfusion. Imaging showed a volume increase (five of five) and a heterogeneous T2-hypointensity of orbital fat (four of five). This preliminary study suggests that swelling and T2-hypointensity of orbital fat may be a marker of global brain swelling and/or increased intracranial pressure.


1998 ◽  
Vol 89 (3) ◽  
pp. 448-453 ◽  
Author(s):  
Ingunn R. Rise ◽  
Ole J. Kirkeby

Object. The authors tested the hypothesis in a porcine model that inhibition of nitric oxide synthesis during reduced cerebral perfusion pressure (CPP) affected the relative cerebral blood flow (CBF) and the cerebrovascular resistance. Methods. The CPP was reduced by inducing high cerebrospinal fluid pressure and hemorrhagic hypotension. With continuous blood and intracranial pressure monitoring, relative CPP was estimated using the laser Doppler flowmetry technique in nine pigs that received 40 mg/kg nitro-l-arginine methyl ester (l-NAME) and in nine control animals. The l-NAME caused a decrease in relative CBF (p < 0.01) and increases in cerebrovascular resistance (p < 0.01), blood pressure (p < 0.05), and CPP (p < 0.001). During high intracranial pressure there were no significant differences between the treated animals and the controls. After hemorrhage, there was no significant difference between the groups initially, but 30 minutes later the cerebrovascular resistance was decreased in the control group and increased in the l-NAME group relative to baseline (p < 0.05). Combined hemorrhage and high intracranial pressure increased the difference between the two groups with regard to cerebrovascular resistance (p < 0.05). Conclusions. These results suggest that nitric oxide synthesis inhibition affects the autoregulatory response of the cerebral circulation after cardiovascular compensation has taken place. Nitric oxide synthesis inhibition enhanced the undesirable effects of high intracranial pressure during hypovolemia.


1985 ◽  
Vol 63 (4) ◽  
pp. 532-536 ◽  
Author(s):  
John R. Ruge ◽  
Leonard J. Cerullo ◽  
David G. McLone

✓ The authors present two cases of pneumocephalus occurring in patients with permanent shunts and review nine previously reported cases. Mental status changes and headache are the most common presenting symptoms. Six of the 11 cases of pneumocephalus occurred in patients with shunt placement for hydrocephalus secondary to aqueductal stenosis. In these patients, thinned cerebrospinal fluid barriers secondary to longstanding increased intracranial pressure may predispose them to pneumocephalus. Temporary extraventricular drainage is an effective method of treatment in this group of patients. Two other etiologies are identified with significance to treatment, and the role of craniotomy is discussed.


1981 ◽  
Vol 54 (1) ◽  
pp. 49-57 ◽  
Author(s):  
Hiroshi Yamada ◽  
Shigetoshi Nakamura ◽  
Masataka Tajima ◽  
Naoki Kageyama

✓ The neurological and neuroradiological manifestations of pediatric achondroplasia are analyzed on the basis of 10 cases. In addition to the classical symptomatology of an enlarging head, with or without increased intracranial pressure, several patients presented symptoms related to a small foramen magnum. Respiratory problems and quadriparesis were also observed in these patients. Six patients who were treated by foramen magnum decompression showed remarkable improvement. Neuroradiological evaluation revealed a significant number of patients with dilated cortical sulci and basal cisterns, and mild dilatation of the ventricles on computerized tomography (CT) and/or ventriculography. Two patients showed signs of obstruction at the fourth ventricle outlets. Some exhibited anomalous dilatation of cerebral veins and dural sinuses, narrowing of the sinuses at the jugular foramen, and enlarged emissary veins. Ventriculoperitoneal or ventriculoatrial shunts were placed in three patients. In the other children with a large head and dilated ventricles, head growth curves paralleled the normal slope, and there was no significant clinical evidence of increased intracranial pressure or enlarging ventricles on follow-up CT scan.


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