Intracranial hypertension

Idiopathic intracranial hypertension as an initial presentation of systemic lupus erythematosus

Authors: Maloney K.

A 14-year-old girl with no known illness presented with a several week history of headaches and vomiting. The patient also reported having joint pain and swelling to the wrists and knees. She had no prior history of headaches, use of hormonal contraception or other medications, recent weight changes or family history of autoimmune disease. Blood pressure temperature, height and weight were normal. She was alert, there was alopecia, cervical lympadenopathy, symmetrical synovitis to the wrists, bilateral papilloedema and cranial nerve VI palsy. Laboratory investigations revealed a normochromic normocytic anaemia, leucopenia and lymphopenia. Serum chemistries were normal. CT of the brain was normal. Lumbar puncture revealed an opening pressure of greater than 300 mm H2O; cerebrospinal fluid (CSF) analysis was normal. HIV antibodies were non-reactive. Despite treatment with acetazolamide she developed somnolence. Hence MR venography was performed which showed no evidence of cerebral vein thrombosis. Further investigations revealed a positive direct coombs test, positive antinuclear antibodies (ANA) positive antidouble-stranded DNA (dsDNA) and false positive VDRL. Complement levels were reduced. Anti-Smith, anticardiolipin antibodies and lupus anticoagulant were negative.

Review and recommendations on management of refractory raised intracranial pressure in aneurysmal subarachnoid hemorrhage

Authors: Mak CH, Lu YY, Wong GK.

Intracranial hypertension is commonly encountered in poor-grade aneurysmal subarachnoid hemorrhage patients. Refractory raised intracranial pressure is associated with poor prognosis. The management of raised intracranial pressure is commonly referenced to experiences in traumatic brain injury. However, pathophysiologically, aneurysmal subarachnoid hemorrhage is different from traumatic brain injury. Currently, there is a paucity of consensus on the management of refractory raised intracranial pressure in spontaneous subarachnoid hemorrhage. We discuss in this paper the role of hyperosmolar agents, hypothermia, barbiturates, and decompressive craniectomy in managing raised intracranial pressure refractory to first-line treatment, in which preliminary data supported the use of hypertonic saline and secondary decompressive craniectomy. Future clinical trials should be carried out to delineate better their roles in management of raised intracranial pressure in aneurysmal subarachnoid hemorrhage patients.

Viral-Induced Intracranial Hypertension Mimicking Pseudotumor Cerebri

Authors: Ravid S, Shachor-Meyouhas Y, Shahar E, Kra-Oz Z, Kassis I.

BACKGROUND: Pseudotumor cerebri or idiopathic intracranial hypertension is characterized by normal spinal fluid composition and increased intracranial pressure in the absence of a space-occupying lesion.
METHODS: This study describes a subgroup of 10 patients with the same typical presenting symptoms (headache, vomiting, and papilledema) but without nuchal rigidity, meningeal signs, or change in mental status. Patients had normal neuroimaging studies and intracranial hypertension but also pleocytosis in the cerebrospinal fluid, suggesting central nervous system infection. From the results it can be hypothesized that those children represent a unique subgroup of viral-induced intracranial hypertension when comparing their risk factors, clinical course, treatment, and outcome with 58 patients who had idiopathic intracranial hypertension.
RESULTS: All patients with viral-induced intracranial hypertension presented with papilledema but none had reduced visual acuity or abnormal visual fields, compared with 20.7% of patients who had idiopathic intracranial hypertension. They also responded better to treatment with acetazolamide, needed a shorter duration of treatment (7.7 ± 2.6 months vs 12.2 ± 6.3 months, P = 0.03), and had no recurrences.
CONCLUSION: The results suggest that children who fulfill the typical presenting signs and symptoms and all diagnostic criteria for pseudotumor cerebri other than the normal cerebrospinal fluid component may represent a unique subgroup of viral-induced intracranial hypertension and should be managed accordingly. The overall prognosis is excellent.
Copyright © 2013 Elsevier Inc. All rights reserved.

Idiopathic intracranial hypertension from the perspective of headache center

Authors: Ljubisavljevic S, Trajkovic JZ, Sternic NC, Spasic M, Kostic V.

Idiopathic intracranial hypertension (IIH) is a pathological state defined as an increase of intracranial pressure in the absence of a causative pathological process. The aim of this study was to evaluate the clinical features of the patients with IIH diagnosed in our Headache Center according to the current knowledge of this disorder. In the retrospective and cross-sectional analysis of 3395 patients we present 12 newly diagnosed IIH patients, ten women and two men, aged from 19 to 51, with obtained values of cerebrospinal fluid pressure between 250 and 680 mm of water. The symptoms of IIH clinical presentation have been headache, reported by 92 % of patients; papilledema, noted in 67 %; and cranial nerve impairment (25 %). The results obtained from presented patients confirmed the presence of headache features that are included in criteria for headache attributed with IIH in majority of them: progressive, daily, diffuse, non-pulsatile headache with aggravation by coughing or straining. Decrease of pain intensity after lumbar puncture was noted in all patients. We notice the relatively small proportion of patients with headache attributed to IIH among the patients treated in our Headache Center. The prevalence of IIH is not low and headache is the most frequent presenting symptom; therefore, we could only conclude that some chronic headache patients refractory for treatment are patients with IIH.

Idiopathic intracranial hypertension and transverse sinus stenoses

Authors: Skyrman S, Fytagoridis A, Andresen M, Bartek J Jr.

An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed to be associated with the IIH. Initial treatment consisted of symptom relief by a temporary lumbar drain for cerebrospinal fluid (CSF) diversion, while the pros and cons of a more permanent solution by insertion of a ventriculoperitoneal shunt (VPS) or bilateral transverse sinus stent was discussed. A VPS was inserted since the patient had improved with CSF diversion. MRI verified reopening of the venous sinuses after shunt placement, and the patient remains asymptomatic with no signs of relapse after 3 years of follow-up.

Spontaneous cerebrospinal fluid leak repair: A five-year prospective evaluation

Authors: Chaaban MR, Illing E, Riley KO, Woodworth BA.

OBJECTIVES / HYPOTHESIS Mounting evidence indicates the majority of spontaneous cerebrospinal fluid (CSF) leaks are associated with intracranial hypertension. The objectives of the current study were to assess outcomes regarding spontaneous CSF leaks focusing on premorbid factors, surgical technique, and management of intracranial pressure.
STUDY DESIGN: Prospective cohort.
METHODS: Prospective evaluation of patients with spontaneous CSF leaks was performed. Data regarding demographics, nature of presentation, body mass index (BMI), location and size of defect, intracranial pressure, clinical follow-up, and complications were collected.
RESULTS: Over 5 years, 46 patients (average age, 51 years) with 56 spontaneous CSF leaks were treated by a single otolaryngologist. Twenty-one subjects presented with recurrence of their CSF leak following previous endoscopic and/or open approaches by other physicians. Obesity was present in 78% of individuals (average BMI, 35.6). Fifty-two CSF leaks (93%) were successfully repaired at first attempt. With secondary repair, all CSF leaks were closed at last clinical follow-up (average, 93 weeks). Three patients developed late failures (>2 months), with one recurrence at a distinct location from the primary site at 8 months postprocedure (associated with ventriculoperitoneal shunt failure). Opening pressures via lumbar puncture averaged 24.3 ± 8.3 cm H2 0, which increased significantly to 32.3 ± 9.0 cm H2 0 (P < .0001) following closure of the skull base defect(s). Management of intracranial hypertension included acetazolamide (n = 23) or permanent CSF diversion (n = 19, including five revisions of failed preexisting shunts).
CONCLUSIONS: Although spontaneous CSF leaks have the highest recurrence rate of any etiology, prospective evaluation demonstrates high success rates with control of intracranial hypertension.
LEVEL OF EVIDENCE: 4. Laryngoscope, 2013.

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