A patient with megaloblastic anaemia and idiopathic intracranial hypertension: Case history

A young woman with idiopathic intracranial hypertension (IIH) was found to have a severe megaloblastic anaemia due to multiple alimentary vitamin deficiencies. After correction of the anaemia the idiopathic intracranial hypertension disappeared.     

Resolution of idiopathic intracranial hypertension after sustained lowering of cerebrospinal fluid pressure

Idiopathic intracranial hypertension(IIH) is a syndrome of headache due to raised intracranial pressure(ICP) where the cerebrospinal fluid(CSF) is normal and there is no alternative pathology on imaging. The aetiology is unknown. This review questions many of the prevailing views regarding aetiology and treatment of IIH. It explores the concept that there is a vicious cycle of fluctuating raised ICP leading to secondary compression of the transverse sinuses and further elevation of ICP. It also raises the question as to whether this vicious cycle could be relieved by prolonged drainage of CSF as seen in Lumbar puncture induced low-pressure headache or alternatively a lumbar drain.     

Static and pulsatile intracranial pressure in idiopathic intracranial hypertension

Objective

The aim of this observational study was to characterize the static and pulsatile intracranial pressure (ICP) in conservatively (medically) treated idiopathic intracranial hypertension (IIH) patients in need of shunt surgery, and also in patients with chronic daily headache (CDH) without visual disturbances.

Methods

The material includes 14 IIH patients and 7 CDH patients in whom ICP was monitored continuously over-night. Static ICP was characterized by mean ICP, pulsatile ICP was characterized by the wave amplitude, rise time, and rise time coefficient.

Results

In the IIH group all 14 had headache and visual disturbances. Mean ICP was high (>15 mmHg) in only 7 patients (50%), while mean ICP wave amplitude was high (≥4 mmHg) in all 14 (100%). All IIH patients were shunted and improved clinically thereafter (i.e., relief from visual disturbances and/or headache). None in the CDH group had high mean ICP or mean ICP wave amplitude, and none were shunted.

Conclusions

In this cohort of 14 conservatively treated IIH patients with lasting and shunt-responsive headache and visual disturbances, the mean ICP wave amplitude was elevated (≥4 mmHg) in all patients despite normal mean ICP (<15 mmHg) in 7 patients (all but one on medication). Therefore, the pulsatile ICP may be more relevant than the static ICP in the diagnostic setting for patients with IIH. Further prospective standardized approaches are warranted.     

Sudden re-opening of collapsed transverse sinuses and longstanding clinical remission after a single lumbar puncture in a case of idiopathic intracranial hypertension. Pathogenetic implications

Abstract The aetiopathogenetic role of sinus venous obstructions carried by most idiopathic intracranial hypertension (IIH) patients is controversial. We report the case of a young woman diagnosed with IIH with papilloedema and narrowing of transverse sinuses, in which lowering of intracranial pressure by a single 20 ml cerebrospinal fluid (CSF) resulted in a strong dimensional increase of the transverse sinuses. Changes were followed by clinical remission and normalisation of optical nerve calibre, maintained after a 2-month follow-up. Our findings indicate that, although secondary to CSF hypertension, venous sinuses compression may have an important role in hypertensive status maintenance. Pathogenetic implications of venous sinus compression by hypertensive CSF in IIH are discussed.     

Nocturnal carbon dioxide monitoring in patients with idiopathic intracranial hypertension

Background

Idiopathic intracranial hypertension may be associated with sleep apnea. This study evaluated the incidence of sleep breathing disorders in patients with idiopathic intracranial hypertension.

Materials and Methods

Overnight respiratory monitoring was performed in 22 untreated patients with idiopathic intracranial pressure diagnosed at a tertiary medical center over a two-year period and 12 sex- and age-matched control subjects. Breathing measures included heart rate, respiratory rate,oxygen saturation, and continuous end-tidal capnography. Sleep quality and daily fatigue were assessed by self-report questionnaires.

Results

Mean age of the study group was 32.6 ± 12.2 years and of the control group, 37.0 ± 12.9 years. Neither group had significant findings of hypoxia or hypercarbia during sleep, and there were no between-group differences in mean carbon dioxide level (patients, 35.8 ± 4.41 mmHg; controls, 37.6 ± 4.38 mmHg; p > 0.02) or minimal oxygen saturation (96.35 ± 1.99% and 5.69 ± 1.71%, respectively; p > 0.02). The study group had significantly more events of apnea (CO₂) per hour of sleep than the control group (1.21 ± 1.38 and 0.92 ± 0.56, respectively; p = 0.02), although values were still within normal range (<5/hr).

Conclusion

Idiopathic intracranial hypertension is not associated with a clinically significant nocturnal breathing abnormality, and hypercarbia is apparently not involved in the pathogenesis. However, it is possible that a subtle increase in paroxysmal sleep apnea (CO₂) events might be sufficient to cause vasodilatation of the cerebral blood vessels, thereby increasing intracranial pressure. Screening for sleep apnea may be appropriate in idiopathic intracranial hypertension patients, and further studies are needed to clarify this issue.     

Familial intracranial hypertension: a case report

We report the cases of a father and his daughter developing idiopathic intracranial hypertension within a few months' of each other.A second daughter is affected by uveitis, while a third daughter presented an episode of visual loss of unknown nature.

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Sommario Viene descritto il caso di padre e figlia con ipertensione intracranica idiopatica. Una seconda figlia è affetta da uveite, ed una terza ha presentato un episodio di calo visivo di causa non nota.

     

Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center

BACKGROUND: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri (PTC), is a condition characterized by increased intracranial pressure without clinical, laboratory or radiologic evidence of an intracranial space-occupying lesion, meningeal inflammation or venous outflow obstruction. Previous population-based studies evaluated the presenting clinical features of IIH in North-Eastern Libya, Iowa, Louisiana, Minnesota, Northern Ireland, Israel and Western Turkey. However, we report the demographic patterns of a metropolitan North American city. METHODS: We performed a retrospective study of all patients with an initial IIH diagnosis and follow-up at the Detroit Medical Center (DMC), Michigan from 1985 to 2002. Medical records of 174 patients were reviewed, with 77 patients fitting the diagnostic criteria. RESULTS: Seventy-seven patients, 71 females (92.2%) and 6 males (7.8%) were identified. Age ranged from 10 to 50 years, with a mean of 34 years. Fifty patients (65%) were African-American and 24 patients (31%) were Caucasian. The most common presentation was isolated headache (28.6%). Nineteen patients (24.7%) were asymptomatic. Obesity was described in 65 of 74 patients (87.8%) evaluated for body dimensions. CONCLUSIONS: We report an increased number of asymptomatic patients after routine ophthalmic examination. Obese females in metropolitan Detroit would benefit from routine eye evaluations to avoid potential visual loss related to papilledema.     

Visual evoked potentials in idiopathic intracranial hypertension

Objective

Visual evoked potentials (VEPs) are sensitive indicators of optic nerve dysfunction. Since visual loss is a serious complication of idiopathic intracranial hypertension (IIH), we measured VEP in an attempt to evaluate quantitatively the optic nerve damage in patients with chronic IIH.

Methods

We examined 20 consecutive IIH patients fulfilling modified Dandy criteria. VEPs were recorded bilaterally and at midline from occipital electrodes using pattern reversal stimuli to each eye separately. The results were compared to norm values used in our laboratory.

Results

VEP response latencies were usually similar in both eyes. They were delayed in nine patients (45%) bilaterally and in two additional patients unilaterally (55% of patients had abnormal responses in at least one eye). VEP amplitudes were not affected. All three patients with reduced visual acuity in both eyes had prolonged VEP latencies, but abnormal records were seen frequently also in cases with normal visual acuity. Repeated examinations 6–12 months later revealed similar results in clinically stable patients and improvement of VEP latency in parallel to clinical improvement in one.

Conclusions

Clinical visual impairment in IIH is probably preceded by prolongation of VEP responses and the latter may be evidence of optic nerve dysfunction due to demyelination.     

Spinal cord astrocytoma presenting as "idiopathic" intracranial hypertension

Increased intracranial pressure is rarely seen in association with spinal tumors. We describe a young, non-obese man who presented with increased intracranial pressure, papilledema and visual obscuration. Multiple cerebrospinal fluid (CSF) examinations with normal or minimally elevated CSF protein lead to the initial diagnosis of idiopathic intracranial hypertension. After a lumboperitoneal shunt placement a progressive thoracic myelopathy developed 7 months after onset of symptoms. The spinal MRI showed a low cervical-upper thoracic intramedullary tumor. Open biopsy confirmed a grade 3 fibrillary astrocytoma. The suspected mechanisms of spinal tumors causing increased intracranial pressure are reviewed as well as three other cases of spinal astrocytomas previously reported in the literature that presented with papilledema and increased intracranial pressure without hydrocephalus. This case illustrates that increased intracranial pressure may in exceptional cases of spinal tumors precede the more typical myelopathic presentation by months and mimic idiopathic intracranial hypertension.     

Rebound intracranial hypertension after treatment of spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache and the cause is usually cerebrospinal fluid leaks in spine level. Most patients with SIH have a benign course. Epidural blood patch (EBP) is the treatment of choice when initial conservative managements are ineffective. We reported a patient with SIH diagnosed by using magnetic resonance imaging and radionuclide cisternography. Acute rebound intracranial hypertension developed after EBP and was successfully treated with intravenous osmotic agent.     

原发性高颅压的垂体及蝶鞍影像学形态变化(附28例分析)

目的　研究原发性高颅压病人垂体和蝶鞍形态变化与病程的关系。方法　回顾性分析原发性高颅压 2 8例病人的临床资料 ,并对垂体和蝶鞍形态变化分别分级。结果　本组垂体形态正常 3例 ,垂体 /垂体窝≥ 50 %者 6例 ,≤ 50 %者 1 0例 ,空蝶鞍 9例。蝶鞍形态正常 1 0例 ,蝶鞍稍扩大 1 2例 ,明显扩大 6例。病程小于 3个月者 ,3例出现垂体形态变化 ;3～ 1 2个月病程者均出现垂体形态改变 ,仅半数有蝶鞍形态改变 ;病程大于 1 2个月者均出现垂体和蝶鞍形态变化。结论　原发性高颅压患者垂体形态变化早于蝶鞍形态变化 ,垂体形态变化明显者的病程长于垂体形态正常或变化较轻者 ,此表现有助于早期提示和诊断原发性高颅压。     

Is there a difference in outcomes of patients with idiopathic intracranial hypertension with the choice of cerebrospinal fluid diversion site: a single centre experience

The visual and headache outcomes in patients with idiopathic intracranial hypertension (IIH) undergoing cerebrospinal fluid diversion with a lumboperitoneal (LPS) or ventriculoperitoneal shunting (VPS) have not been well reported. The aims of this study were to: (a) to assess outcomes of CSF diversion in IIH, (b) to understand influence of the type of shunt in outcomes, and (c) to understand factors predisposing in shunt failure.

Methods

The medical records of 34 patients who underwent cerebrospinal fluid diversion (CSFD) between 1996 and 2007 were retrieved and epidemiological and clinical data was collected.

Results

The mean age was 35 (±7.9) years. Thirty-four patients underwent 63 shunt placements in total. 85% follow-up was achieved. The mean follow-up for the entire group was 28.9 (±31.8) months. Headaches improved more than visual disturbances. There was no significant difference between the groups that received a VPS and those receiving an LPS in both headache and visual outcomes. The rate of complications was 20.5% and the need for revision was 35% for the whole group. Patients with LPS suffered more complications and first time revisions than patients with VPS. No factor recorded could predict the need for revision or final outcomes. The shunts of patients receiving a VPS tend to survive longer than those receiving primarily an LPS, however the difference is not statistically significant.

Conclusions

Predicting which patients will improve is not possible at present. The influence of site diversion is not critical but patients with VPS have less complications and revisions than those receiving a LPS.     

Ventriculoperitoneal shunt treatment in a pregnant renal transplant recipient with idiopathic intracranial hypertension: Case report and review of the literature

Idiopathic intracranial hypertension (IIH) is a relatively uncommon disorder characterised by raised intracranial pressure without an established pathogenesis. Diagnosis of IIH requires the demonstration of symptoms and signs referable only to elevated intracranial pressure; cerebrospinal fluid (CSF) opening pressure >25 cm H₂O measured in the lateral decubitus position; normal CSF composition; and no evidence for an underlying structural cause demonstrated by using MRI or contrast-enhanced CT scan for typical patients and MRI and MR venography for atypical patients such as man, children and those with low body mass index. We present a 38-year old primigravid renal transplant patient at 7 weeks of gestation who presented with 2 weeks of intense, throbbing, holocranial headache, nausea, vomiting, photophobia, diplopia and progressive visual loss. When medical treatment fails and/or not appropriate to use due to the reported of teratogenic risks in pregnant women, surgical interventions gain importance. In this particular patient, venticuloperitoneal shunt was chosen as the CSF diversion technique. In this case report indications, contraindications in addition to outcomes regarding headache, vision loss and the resolution of papilloedema of the present surgery options for IIH are discussed.     

Venous obstruction and jugular valve insufficiency in idiopathic intracranial hypertension

The pathophysiology of elevated intracranial pressure in idiopathic intracranial hypertension (IIH) is unclear. Cerebral venous outflow obstruction and elevated intracranial venous pressure may play an etiological role. We examined jugular valve insufficiency as a potential factor contributing to intracranial hypertension. Jugular venous valve function was assessed bilaterally by duplex sonography in 20 consecutive patients with diagnosis of IIH and in 20 healthy controls matched for age, gender and body mass index. Diagnosis of valvular insufficiency was based on reflux duration during a controlled Valsalva maneuver. Intracranial venous outflow was evaluated in 11 patients (MR venography in 10, digital subtraction angiography (DSA) in two cases). As a principle result, valvular insufficiency was significantly more frequent in patients with IIH (70 vs. 30%; p < 0.05). This finding was associated with irregular leaflet structures on B-mode imaging (p < 0.01). Bilateral insufficiency was more frequent in the patient group which, however, was not significant (p = 0.08). In addition, sinovenous outflow obstruction was found in five of six patients that had undergone contrast-enhanced MR venography and DSA. The detection rate was inferior in phase-contrast MR imaging (one of five patients). In conclusion, this study gives evidence that valvular insufficiency may play a causal role in IIH. Obesity is a major risk factor for the disease and weight reduction leads to improvement of symptoms. Possibly, increased intra-abdominal pressure is transmitted into the intracranial venous system, causing intracranial hypertension. Jugular valve insufficiency may facilitate pressure transmission. As transverse sinus stenosis was a concomitant finding, these factors may be complementary.     

The incidence and prevalence of idiopathic intracranial hypertension in Sheffield,UK

Background and purpose: The purpose of this study was to identify the incidence and prevalence of idiopathic intracranial hypertension (IIH) in Sheffield, UK. Methods: A retrospective review of case notes was conducted to identify cases of IIH seen between 1 January 2007 and 31 December 2008. Results: Sixteen (15 women and 1 man) new patients were identified to give an incidence within Sheffield of 1.56/100 000/year and 2.86/100 000/year for women. The incidence of IIH in obese women was 11.9/100 000/year. The prevalence of IIH was calculated as 10.9/100 000, and 85.7/100 000 in obese women. Conclusion: A higher incidence of IIH than previously reported UK data was found, which may be because of increasing obesity within the population, or improved case ascertainment.     

Pseudotumor cerebri/idiopathic intracranial hypertension in children: An experience of a tertiary care hospital

Objective: Pseudotumor cerebri (PTC) is diagnosed at increasing rates probably due to the increase in obesity prevalence all over the world and awareness about the disease. Our aim in this study was to evaluate the PTC clinical picture and etiological factors in children at the present time. Method: The records of 53 patients with 32 females, who were diagnosed with PTC in a child neurology department between the years of 2005 and 2012 were retrospectively analyzed. Results: The mean age at presentation was 10.9 years (3–17 years) and approximately half of patients were aged of 11 years or less. While more than half of prepubertal patients were male, girls rate reaches 74% at puberty. An etiological factor such as venous sinus thrombosis, infections, anemia, steroid discontinuation, drugs, slit ventricle syndrome and minor head injury causing the PTC was identified in 43% of the patients. The mean duration of treatment was 6.4 months (3–24 months) and the mean follow-up duration 16.5 months (3–52 months). Visual field constriction was moderate in only two pubertal and obese female patients and mild in four patients. Conclusions: PTC is seen in prepubertal children as often as in puberty. An etiological factor causing PTC is present in about half the patients in childhood. The main etiological factors of the disease currently consist of cranial venous thrombosis, infections, anemia and drugs. Malnutrition, renutrition and related vitamin deficiencies or excesses commonly seen previously have become less important in PTC etiology. PTC is a disease that requires long-term treatment and follow-up but the prognosis is good in patients who are diagnosed early, receive appropriate treatment and show good compliance with the treatment.     

Long-term visual outcome in idiopathic intracranial hypertension

Objective:

To characterize the course, outcome, and risk of relapse or late worsening in a clearly defined cohort of idiopathic intracranial hypertension (IIH) over a long period of follow-up.

Materials and Methods:

Retrospective chart review of patients with definite IIH was evaluated at the Sree Chitra Tirunal Institute for Medical Sciences and Technology between 1998 and 2006. Patients’ demographic data, clinical, neuro-ophthalmic examinations, and treatment details were abstracted. Patients were further categorized into three groups based on whether they improved, worsened, or relapsed on follow-up. Final visual outcome of each patient was defined according to grading of the worse eye at the last visit. Statistical analysis included t test to compare group means and chi-square test to compare proportions.

Results:

Of the 43 women included, visual impairment was observed in 80 eyes (93%) at presentation and it was moderate to severe in 14%. The mean CSF opening pressure at presentation did not differ significantly in those with visual impairment compared to those with normal vision. Those having early severe visual impairment had significantly higher (P = 0.015) likelihood of severe visual impairment on last follow-up. Of the total, 34 patients (79%) improved, 4 (9.3%) relapsed on follow-up after period of stability, and 5 (11.6%) worsened over 56 months follow-up (range, 26-132 months). The groups were comparable, except those who improved were younger (P<0.05). At last examination, 9% had significant vision loss.

Conclusion:

IIH patients can have delayed worsening or relapses and about tenth of patients can have permanent visual loss early or late in the course of the disease. All patients with IIH need to be kept under long-term follow-up, with regular monitoring of visual functions.