Familial benign intracranial hypertension.

Three sisters with benign intracranial hypertension are reported. This is the first documentation of benign intracranial hypertension in three family members. Obesity is a striking feature in these patients as well as five of the six previously reported patients with familial benign intracranial hypertension. Pregnancy and chronic dysfunctional uterine bleeding, well known predisposing factors in this syndrome when it occurs sporadically, were present in two of the sisters. A familial metabolic defect may be responsible for the intracranial hypertension in these patients.     

Benign intracranial hypertension: atypical presentation of Miller Fisher syndrome?

Acute ocular paresis, nausea, vomiting, and headaches associated with high intracranial pressure without obvious intracranial pathology are typical features of benign intracranial hypertension. We describe two young children whose presentation, initially suggestive of idiopathic or benign intracranial hypertension, evolved to comprise ophthalmoplegia, ataxia, and areflexia. This triad characterizes Miller Fisher syndrome, a clinical variant of Guillain-Barré syndrome that occurs rarely among children. In both patients, this diagnosis was supported by the clinical course and neurophysiologic findings. Plasma serology was positive for Campylobacter jejuni and anti-GQ1b antibodies in one patient and for antimyelin antibodies in the other. This report of two children with Miller Fisher syndrome presenting with intracranial hypertension adds to the findings for a similar patient treated previously, which raises the question concerning the possible role or contribution of benign intracranial hypertension in Miller Fisher syndrome.     

Cerebrospinal fluid neurohypophysial peptides in benign intracranial hypertension.

The cerebrospinal fluid (CSF) concentrations of arginine vasopressin (AVP) and oxytocin (OT) were investigated both in patients with benign intracranial hypertension and in age and sex matched controls. Twenty eight lumbar punctures were performed on 15 patients with benign intracranial hypertension as part of their routine investigation and therapy. All patients had raised intracranial pressure (27.4, SE 1.7 cm.CSF). CSF AVP levels were significantly elevated in benign intracranial hypertension (2.1, SE 0.3 pmol/l) compared with controls (0.7, SE 0.1 pmol/l, p less than 0.001) but CSF OT concentrations were similar in both groups. CSF osmolality and plasma AVP and osmolality were identical in patients and controls. There was no correlation between CSF AVP concentration and intracranial pressure. The selective elevation of AVP in CSF may be of importance in the pathogenesis of raised intracranial pressure in benign intracranial hypertension.     

Is "benign intracranial hypertension" really benign?

Hypothalamic-hypophyseal insufficiency has been found in seven of eight patients with so-called benign intracranial hypertension, of whom four showed an inadequate adrenal response to stress. The syndrome of benign intracranial hypertension cannot therefore be considered entirely benign and patients should receive full endocrinological assessment and follow up.     

Increased cerebral blood volume in benign intracranial hypertension.

In two patients with benign intracranial hypertension, the regional cerebral blood volume was markedly elevated (mean of 85 percent) while regional cerebral blood flow was slightly reduced (mean of 10 percent). Reduction of cerebrospinal fluid pressure by removal of cerebrospinal fluid reduced the mean values of regional cerebral blood volume by 13 percent without significant change in regional cerebral blood flow. The abnormal regional volume and regional flow returned to normal concurrent with the clinical improvement. Vinous engorgement and increased intracranial blood volume appear to play an important part in the pathophysiology of increased intracranial pressure in benign intracranial hypertension. A unified concept of the pathogenesis of benign intracranial hypertension is proposed.     

Mean intracranial pressure monitoring by a non-invasive audiological technique: a pilot study.

Intracranial pressure is normally transmitted to the perilymph of the cochlea via the cochlear aqueduct. The relationship between perilymphatic pressure, indirectly measured by tympanic membrane displacement, and mean intracranial pressure defined either clinically or by direct measurement has been examined in 58 patients (aged 5-77 years), with hydrocephalus, benign intracranial hypertension, intracranial tumours, subarachnoid haemorrhage and head injuries. The most consistent results were obtained in young patients with hydrocephalus and benign intracranial hypertension. However, the technique was not suitable when the stapedial reflex was absent as a result of middle ear/brainstem dysfunction and did not reflect intracranial pressure when the cochlear aqueduct was not patent. This pilot study suggests that the tympanic membrane displacement technique may provide a useful non-invasive method for serial monitoring of intracranial pressure in young patients with hydrocephalus or benign intracranial hypertension.     

Benign intracranial hypertension: a cause of CSF rhinorrhoea.

Four patients undergoing treatment for benign intracranial hypertension presented with spontaneous CSF rhinorrhoea. The four patients, all women, were aged between 33 and 44 years. They had been receiving treatment for benign intracranial hypertension for a period ranging from eight months to 11 years, before developing the CSF leak. There was no history of previous head injury and there were no congenital anomalies of the floor of the anterior fossa. The site of the CSF fistula was localised to the cribriform plate in all four cases. The pathophysiology of the CSF rhinorrhoea and the surgical management of this group of patients are discussed. The authors propose that benign intracranial hypertension should be included in the classification of high pressure CSF leaks.     

Benign intracranial hypertension associated with budesonide treatment in children with Crohn's disease.

Oral budesonide in adult studies is a potent corticosteroid with decreased systemic bioavailability and an improved adverse effect profile in comparison with prednisone. It has recently been introduced for the treatment of inflammatory bowel disease in Europe, Canada, and Israel. Benign intracranial hypertension has rarely been associated with corticosteroid therapy but has not been reported in association with budesonide therapy. Three adolescents with Crohn's disease and poor nutritional status developed benign intracranial hypertension while receiving oral budesonide. All three patients had previously received multiple courses of prednisone during the course of their disease, without developing intracranial hypertension. Benign intracranial hypertension resolved after medication withdrawal and did not recur with subsequent use of prednisone. Evaluation for benign intracranial hypertension should be considered in patients with inflammatory bowel disease who develop headache while receiving oral budesonide. This side effect may be associated with poor nutritional status.     

Vitamin A induced benign intracranial hypertension.

A case of benign intracranial hypertension due to prolonged administration of a low dose of Vitamin A is described in a young male investigated as a brain tumor suspect. Computerized axial tomography showed small and symmetrical lateral ventricles which was consistent with the diagnosis. The syndrome of benign intracranial hypertension and its relationship to chronic Vitamin A toxicity is discussed.     

Benign intracranial hypertension: visual loss and optic nerve sheath fenestration.

Five patients with serious ocular complications of benign intracranial hypertension are described. Optic nerve sheath fenestration resulted in resolution of papilloedema with, in three instances, improvement in vision. Benign intracranial hypertension may not always be benign for vision and fenestration operations may prevent or reverse visual deterioration by an effect on the optic nerve rather than by reducing intra-cranial pressure.     

Serial ventricular volume measurements: further insights into the aetiology and pathogenesis of benign intracranial hypertension.

Ventricular volumes have been measured from CT scans of patients with benign intracranial hypertension both at initial presentation and at a later date. Volumes initially were smaller than normal, but at review five patients showed a significant increase in ventricular size. Persisting small ventricular volume correlated with persisting symptoms and signs and with persisting obesity. This supports the view that patients with benign intracranial hypertension have brain swelling and that obesity may be implicated in the pathogenesis, perhaps via a neuroendocrine disturbance. It is suggested that weight reduction may be an important component of treatment.     

Regional cerebral oxygen utilization, blood flow, and blood volume in benign intracranial hypertension studied by positron emission tomography

Using PET, we measured regional cerebral oxygen utilization, oxygen extraction, blood flow, and blood volume in five patients with benign intracranial hypertension. No significant differences in regional cerebral function were found between the patients and 15 age-matched normal controls. Cerebral decompression with a lumboperitoneal shunt produced little change in regional cerebral function in one patient studied serially. The raised CSF pressure of benign intracranial hypertension is therefore not associated with any significant deterioration in cerebral oxygen metabolism or hemodynamics.     

The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension

Of 101 patients with benign intracranial hypertension not related to vasculitis, neck stiffness occurred in 31, tinnitus in 27, distal extremity paresthesias in 22, joint pains in 13, low back pain in 5, and gait "ataxia" in 4. Symptoms resolved promptly upon lowering the intracranial pressure by lumbar puncture, and were probably directly caused by intracranial hypertension. Awareness of these "minor" symptoms of increased intracranial pressure can facilitate diagnosis and management.     

Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow in patients with benign intracranial hypertension (pseudotumor cerebri)

Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow were investigated in 14 patients with benign intracranial hypertension (pseudotumor cerebri). Intracranial pressure was increased in 9 patients (20 to 30 mm Hg), borderline in 4 patients (15 to 18 mm Hg), and normal in 1 patient (8 mm Hg). Six patients had plateau waves, and all had B waves in more than 50% of the monitored time. Conductance to cerebrospinal fluid outflow, measured by a lumbo-lumbar perfusion method, was significantly reduced: 0.042 ml X mm Hg-1 X min-1 (+/- 0.004 [SEM]; normal, more than 0.080 ml X mm Hg-1 X min-1). Cerebral blood flow was measured by xenon 133 inhalation and single photon emission computer tomography. Mean hemispheric flow was normal in all cases, averaging 59 +/- 9 ml X 100 gm-1 X min-1. Only 2 patients showed focal low-flow areas. Thus, a disturbance of cerebrospinal fluid circulation seems to be of pathogenetic significance in benign intracranial hypertension.     

Langerhans' cell histiocytosis presenting as intracranial hypertension.

Benign intracranial hypertension is known to be associated with obesity, endocrine abnormalities, various medications, and cerebral venous sinus thrombosis. We report a patient presenting with headaches and vomiting attributed to benign intracranial hypertension. The diagnostic work-up revealed Langerhans' cell histiocytosis of the occipital bone. There was no evidence for cerebral vein thrombosis by cranial computed tomography scan, Doppler ultrasonography, planar and single photon emission computed tomography technetium 99m-labelled red blood cell scintigraphy, and magnetic resonance angiography. Excision of the occipital bone lesion and a short course of acetazolamide and prednisone were curative. We hypothesize that cytokines secreted by the tumor were responsible for the development of intracranial hypertension.     

Pseudotumor cerebri.

Probable cases of pseudotumor cerebri were described more than a hundred years ago. A great deal of controversy existed regarding proper terminology and disease characterization. The clinical entity was doubtful before the routine use of ventriculography in the 1930s. Throughout history, many terms have been used, including serous meningitis, pseudotumor cerebri, otitic hydrocephalus, angioneurotic hydrocephalus, toxic hydrocephalus, meningeal hypertension, hypertensive meningeal hydrops, pseudoabscess, intracranial hypertension of unknown cause, papilledema of indeterminate etiology, intracranial pressure without brain tumor, benign intracranial hypertension, and idiopathic intracranial hypertension.     

EEG in benign intracranial hypertension

A retrospective study of standard EEG's obtained from 26 patients with benign intracranial hypertension was done with particular emphasis on the effect of age on the EEG findings. Abnormal EEG's were found in 10 patients, all were less than 20 years of age. Although no consistent pattern of EEG abnormality could be identified, there appears to be a difference between the immature and adult brain in the EEG response to intracranial hypertension.     

Raised intracranial pressure in minimal forms of craniosynostosis

Most cases of craniosynostosis are diagnosed during early infancy, but occasionally craniosynostosis evolves with minimal cranial involvement and goes unnoticed until late childhood. Seemingly these mild forms of craniosynostosis cause few, if any, symptoms of neurological involvement. We describe the cases of a 9-year-old girl and a 6-year-old boy who presented with evident signs of raised intracranial pressure (ICP), together with a negligible skull deformity. We have termed these cases as occult craniosynostosis. Differential diagnosis in our patients was established against known causes of benign intracranial hypertension. Bilateral expanding craniotomies afforded total relief from the symptoms and signs of raised ICP. Neurosurgeons treating children with symptoms and signs of benign intracranial hypertension should be aware of the possibility of minimal forms of craniosynostosis evolving with marked manifestations of raised ICP. Received: 18 May 1998     

Arginine vasopressin levels in cerebrospinal fluid in neurological disease

Levels of arginine vasopressin have been measured in the blood and cerebrospinal fluid of patients with benign intracranial hypertension and raised intracranial pressure, patients with other neurological diseases and in normal control subjects. There was no difference in blood levels in each of the 3 groups (mean ± SEM, 2.8 ± 0.5, 2.5 ± 0.25, 2.53 ± 0.4 pg/ml). However, levels of arginine vasopressin in the cerebrospinal fluid in patients with benign intracranial hypertension and other neurological diseases were higher (mean ± SEM, 0.64 ± 0.05, 0.61 ± 0.04 pg/ml), than in the control group (0.49 ± 0.06), but not different from each other. The origin of arginine vasopressin in cerebrospinal fluid is uncertain and a number of possibilities are discussed.     

An unusual case of superior sagittal sinus thrombosis accompanied with dural AV fistula]

Benign intracranial hypertension or pseudotumor cerebri is an collective term for a number of diverse syndromes characterized by increased intracranial pressure. Neither intracranial mass nor ventricular dilatation is observed in this disorder. Moreover, the pathogenesis of this syndrome has yet to be determined. We report a case of 36-year-old female diagnosed as benign intracranial hypertension, who has developed superior sagittal sinus thrombosis and dural AV fistula during the follow up period. The patient was pointed out to have papilledema and elevated intracranial pressure six years ago. Although she was examined by both DSA and CT scan, no abnormal intracranial lesions were observed. Consequently, she was diagnosed as the benign intracranial hypertension and had been followed as an out patient. Three years later, lumboperitoneal shunting was performed because of severe headache and visual impairment. Postoperatively, the patient had been well for two years. Recently, occipital headache recurred and she was readmitted to our hospital. MRI studies demonstrated dilated vessels in the right occipital area. Additionally, angiograms revealed not only the superior sagittal sinus thrombosis but also the rich network of dural AV fistula adjacent to the occlusion. According to those results, the superior sagittal sinus was supposed to have the incomplete occlusion or delayed blood flow that were not observed by DSA, MRI and CT scan performed previously. Those occlusive change in the superior sagittal sinus impeded the CSF absorption and elevated the pressure of venous inflow, then the arterio-venous communication has been developed.(ABSTRACT TRUNCATED AT 250 WORDS)