Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension

In patients presenting with intracranial hypertension without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition (pseudotumor cerebri syndrome), the diagnosis of intracranial sinus venous thrombosis (ISVT) has crucial etiological, therapeutic and prognostic implications. Utilizing two well-defined groups of pseudotumor cerebri patients, one with magnetic resonance imaging (MRI) or angiography confirmed ISVT (17 patients) and the other in whom ISVT has been excluded (idiopathic intracranial hypertension [IIH], 27 patients), we investigated the characteristics that might be helpful in distinguishing them. No clinical or auxiliary findings differed between the ISVT and IIH groups except for female gender and lower CSF protein level, which were significantly associated with the latter. While the syndrome pseudotumor cerebri could be due to multiple causes including ISVT, the term IIH should be restricted for patients with isolated intracranial hypertension attributable to no other neurological or systemic disease. Since CT frequently misses ISVT, patients with pseudotumor cerebri syndrome should undergo MRI and MR venography before being labeled as IIH. We conclude that Modified Dandy's Diagnostic Criteria of pseudotumor cerebri, formulated prior to MRI era, can no longer be applied for the diagnosis of IIH.     

Hydrocephalus caused by increased intracranial venous pressure: A clinicopathological study

A child developed progressive communicating nonobstructive hydrocephalus as a result of increased intracranial venous pressure. The child had been treated for congenital heart disease at age 3 weeks by surgical creation of an anastomosis between his superior vena cava and right pulmonary artery. Although his cardiac symptoms were alleviated, intracranial venous hypertension resulted, giving rise to progressive head enlargement and other signs of hydrocephalus. Postmortem examination at age 3 years disclosed no other lesions that could have caused the hydrocephalus. Increased intracranial venous pressure can lead to either pseudotumor cerebri or hydrocephalus, the former in children 3 years or older, the latter in infants 18 months or less. Possible mechanisms accounting for these differences are discussed.     

Idiopathic intracranial hypertension in the pediatric population

The aim of this study was to determine the features of pseudotumor cerebri or idiopathic intracranial hypertension in prepubertal and pubertal children. We retrospectively reviewed patient charts of those 16 years and younger, diagnosed with pseudotumor cerebri/idiopathic intracranial hypertension. Our study group consisted of 27 patients; the mean age was 10.9 years, and there was a male-to-female ratio of 13 to 14. In the prepubertal group (n = 13), the male-to-female ratio was 8 to 5; in the pubertal group, (n = 14), the ratio was 5 to 9. Overweight or obesity was found in 16 (59%) patients. Outcome was favorable except for one who remained symptomatic. Pseudotumor cerebri/idiopathic intracranial hypertension in children is rare. Its characteristics differ from adults. We found the prepubertal group to be a distinct group since pseudotumor cerebri/idiopathic intracranial hypertension did not occur predominantly in females and was not associated with obesity.     

New developments in idiopathic intracranial hypertension

This review highlights recent additions to the literature regarding the diagnosis, evaluation, and management of idiopathic intracranial hypertension (pseudotumor cerebri). Unique features of pediatric pseudotumor cerebri are addressed as well.     

Diagnostic criteria for idiopathic intracranial hypertension

The syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal CSF composition, previously referred to as pseudotumor cerebri, is a diagnosis of exclusion now termed idiopathic intracranial hypertension (IIH). Diagnostic criteria of this disorder have not been updated since the Modified Dandy Criteria were articulated in 1985. Since then, new developments, including advances in neuroimaging technology and recognition of additional secondary causes of intracranial hypertension, have further enhanced the ability to diagnose conditions that may mimic IIH. These factors are not addressed in the Modified Dandy Criteria. This report describes updated diagnostic criteria for IIH that may be used for routine patient management and for research purposes.     

Pseudotumor Cerebri associated with Hypovitaminos is A and Hyperthyroidism

Pseudotumor cerebri, or benign intracranial hypertension, is relatively rare in children. A 12-year-old girl with Hashimoto hyperthyroidism and hypovitaminosis A is described, who fulfilled the diagnostic criteria for pseudotumor cerebri.     

Stimulant drugs and academic performance in hyperactive children

Small vascular malformations of the central nervous system are generally considered cryptic, or silent, because they are an incidental but frequent finding at autopsy. When they are symptomatic, these malformations have been associated with intracranial hemorrhage or seizures. The patient reported here had multiple small cavernous angiomas associated with a syndrome resembling pseudotumor cerebri. The increased intracranial pressure apparently was produced by multifocal areas of cerebral edema without associated hydrocephalus.     

Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri.

A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. Cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension.     

Pseudotumor cerebri secondary to subacute sclerosing panencephalitis

Pseudotumor cerebri is characterized by signs of raised intracranial pressure occurring in the absence of obvious brain pathology. We present an 8-year-old boy with pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The reported patient suggests a possible association between subacute sclerosing panencephalitis and pseudotumor cerebri and that subacute sclerosing panencephalitis should be added to the list of disorders known to be associated with pseudotumor cerebri. Patients with subacute sclerosing panencephalitis might benefit from treatment aimed at increased intracranial pressure.     

Clinical spectrum of the pseudotumor cerebri complex in children

Purpose  

Our aim was to improve diagnosis and management of pseudotumor cerebri (PTC; also known as idiopathic intracranial hypertension) in children.     

Pseudotumor cerebri. A theory on etiology and pathogenesis

Ten patients with pseudotumor cerebri were studied. The diagnosis was based on bilateral papilledema and absence of intracranial masses and hydrocephalus. Based on the radiological findings, three groups of patients emerged: (A) Five patients with an empty sella. Four had an enlarged sella; (B) Two patients with a pituitary adenoma; and (C) Three patients without sellar or pituitary pathology. The onset of symptoms was acute in four patients, two from group A and one each from groups B and C. One patient from group A had a lesion of the chiasma. Two patients from group B and two from group C had symptoms and signs suggesting engagement of structures in the cavernous sinus. It is suggested that pseudotumor cerebri may be caused by bilateral compression of the cavernous sinuses because of acute necrotic swelling of a preexisting pituitary adenoma.     

Idiopathic intracranial hypertension: relation of age and obesity in children

The relation between obesity and age in children with idiopathic intracranial hypertension (pseudotumor cerebri) has remained uncertain. The authors reviewed the records of 45 consecutive children with newly diagnosed idiopathic intracranial hypertension seen at two medical centers. Forty-three percent of patients aged 3 to 11 years were obese, whereas 81% of those in the 12- to 14-year age group and 91% of those in the 15- to 17-year age group met criteria for obesity (p = 0.01). Younger children with idiopathic intracranial hypertension are less likely to be obese than are older children or adults.     

Management of pediatric patients with pseudotumor cerebri

Introduction  

The care of patients with pseudotumor cerebri (idiopathic intracranial hypertension) involves ophthalmologists, neurologists and neurosurgeons. Its clinical characteristics in the pediatric population are distinct from those in adult patients.     

Pseudotumor Cerebri in Lyme Disease: A Case Report and Literature Review

Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. Serum and cerebrospinal fluid Lyme antibodies were positive. Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.     

Pseudotumor cerebri due to intracranial arteriovenous malformation

The authors present a case history of a meningeal arteriovenous malformation, presenting as a pseudotumor cerebri syndrome. Subarachnoid hemorrhage did not occur and brain configuration was normal on CT-scan. The pathogenesis of papilledema in this case is discussed. Probably the cause must be sought in the massive overload of the venous return, resulting in increase of cerebral blood volume, impairment of CSF absorption and increase of CSF production. The common denominator and final pathway in intracranial hypertension, also directly responsible for papilledema, is CSF hypertension.     

Visual evoked potentials in pseudotumor cerebri

Pattern reversal visual evoked potentials in 13 patients with pseudotumor cerebri were significantly delayed (99 +/- 7.3 ms [mean +/- SD]) compared with the findings in 20 normal subjects (94 +/- 2.7 ms), although only four patients had latencies outside the normal range. There was, however, a significant correlation between the intracranial pressure and the latency of visual evoked potentials. After medical treatment of the intracranial hypertension, visual evoked potential latencies decreased in patients who recovered and in whom the papilledema disappeared. In one patient with progressive visual failure, the visual evoked potentials were abnormal before disturbances of visual fields and visual acuity were evident. Repeated examinations of visual evoked potentials might be of value in patients with pseudotumor cerebri to ensure neurosurgical intervention in due time to prevent visual loss in patients with impending optic nerve atrophy.     

Pseudotumor cerebri associated with obstructive nephropathy

We report two infants with pseudotumor cerebri associated with renal disease. The pathogenesis of increased intracranial pressure in this clinical setting is unclear, but may be mediated by one or more of the conditions commonly associated with pseudotumor cerebri, including sinus thrombosis, increased intravascular fluid volume, anemia, and endocrine disturbances resulting in abnormal calcium and phosphorus metabolism. The onset of pseudotumor cerebri also may be related to changes in vasopressin levels that affect brain water permeability.     

Sutural diastasis caused by pseudotumor cerebri

The diagnosis of pseudotumor cerebri is based on the triad of: (1) papilloedema, (2) elevated intracranial pressure with a normal cerebrospinal constituency and (3) normal central nervous system imaging studies. However, the diagnosis of pseudotumor cerebri is not always straightforward. We report a 19-year-old morbidly obese girl who developed pseudotumor cerebri associated with sutural diastasis of the cranium based on the direct radiographies. Following a ventriculo-peritoneal shunt operation, we demonstrated the closing and perisuturally sclerosis on all major cranial sutures. A careful examination of direct radiographies might be critical for diagnosis of pseudotumor cerebri. Prompt recognition, evaluation, and treatment are needed to prevent permanent visual loss.     

Idiopathische intrakranielle Hypertension

This review describes the clinical findings as well as thes diagnostic and therapeutic options for idiopathic intracranial hypertension (pseudotumor cerebri). Furthermore, the pathophysiological concepts are discussed. Idiopathic intracranial hypertension is characterized by signs and symptoms of raised intracranial pressure with no established pathogenesis. Common symptoms include headaches, visual loss and pulsatile tinnitus. Treatment has two major goals: the alleviation of headaches and the preservation of vision. Weight loss and acetazolamide are the cornerstones in the treatment of the disorder. Drainage of cerebrospinal fluid, optic nerve sheath fenestration and stent angioplasty of a sinus stenosis can be employed in severe cases.