Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

2019 ◽  
Vol 34 (13) ◽  
pp. 806-814 ◽  
Author(s):  
Sama Boles ◽  
Claudia Martinez-Rios ◽  
Daniel Tibussek ◽  
Daniela Pohl
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Treatment Guidelines ◽  
Fluid Pressure ◽  
Specific Treatment ◽  
Unknown Etiology ◽  
Age Group ◽  
Review Of The Literature

Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cerebrospinal fluid studies revealed normal results. The patient’s symptoms transiently resolved after the initial lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmH2O and led to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date. A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical idiopathic intracranial hypertension–related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young age group.

scholarly journals P.074 Infantile idiopathic intracranial hypertension - a case study and review of the literature

2019 ◽  
Vol 46 (s1) ◽  
pp. S34
Author(s):  
S Boles ◽  
D Tibussek ◽  
D Pohl
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Treatment Guidelines ◽  
Specific Treatment ◽  
Pseudotumor Cerebri ◽  
Unknown Etiology ◽  
Visual Deficits ◽  
Ct Head

Background: Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is an increase in intracranial pressure due to unknown etiology. Presentation in infancy is extremely rare. Little is known about infantile IIH and age-specific treatment guidelines are lacking. Methods: Patient data was obtained from medical records at the Children’s Hospital of Eastern Ontario. A literature review of infantile IIH was performed. Results: A previously healthy 9-month-old boy presented with irritability, decreased appetite, and a bulging fontanelle. CT head imaging and cerebrospinal fluid studies revealed normal results. Symptoms transiently resolved after a lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cm H2O and led to a diagnosis of IIH in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/day was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide was increased to 38 mg/kg/day, with no further relapses to date. Conclusions: A diagnosis of IIH is challenging in infants, since the patients cannot yet verbalize typical IIH-related symptoms, and papilledema is difficult to assess. If undetected and untreated, IIH may result in permanent visual deficits.

Lumbar puncture rapidly improves olfaction in patients with idiopathic intracranial hypertension: A cohort study

Cephalalgia ◽  
2020 ◽  
Vol 40 (5) ◽  
pp. 429-436 ◽  
Author(s):  
Nils J Becker ◽  
Sören Enge ◽  
Katharina Maria Kreutz ◽  
Felix Schmidt ◽  
Lutz Harms ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Fluid Pressure ◽  
Olfactory Function ◽  
Clinical Feature ◽  
Healthy Controls ◽  
Cerebrospinal Fluid Drainage ◽  
Olfactory Impairment

Background A lumbar puncture constitutes an important diagnostic procedure in the evaluation of idiopathic intracranial hypertension. Chronic overflow of cerebrospinal fluid into the sheaths of the olfactory nerves appears to be related to olfactory impairment in these patients. Here, we asked whether cerebrospinal fluid drainage in idiopathic intracranial hypertension patients improves olfactory function. Methods Fourteen idiopathic intracranial hypertension patients and 14 neurologic control patients were investigated before and after lumbar puncture using the extended Sniffin’ Sticks procedure. We assessed odor threshold, discrimination, and identification. In idiopathic intracranial hypertension patients, cerebrospinal fluid was drained until cerebrospinal fluid pressure had normalized. In addition, a third group of 14 healthy controls participated in the two smell tests at similar intervals. Results Relative to healthy controls, threshold, discrimination, and identification composite scores before lumbar puncture were significantly lower in idiopathic intracranial hypertension patients and also in neurologic controls. Following lumbar puncture, threshold, discrimination, and identification scores for neurologic controls remained unchanged whereas idiopathic intracranial hypertension patients showed robust improvement on the composite score as well as on all three subscores (all changes: p < 0.003), quickly regaining olfactory function in the normal range. Cerebrospinal fluid opening pressure was significantly correlated with improvement in threshold, discrimination, and identification score upon cerebrospinal fluid drainage ( r = 0.609, p = 0.021). Conclusion Olfactory impairment is an important, yet underappreciated, clinical feature of idiopathic intracranial hypertension. Lowering of increased intracranial pressure improves hyposmia. Our findings shed new light on the pathophysiology of cerebrospinal fluid circulation in idiopathic intracranial hypertension.

scholarly journals Neuro-ophthalmological manifestations after intramuscular medroxyprogesterone: a forme fruste of idiopathic intracranial hypertension?

2016 ◽  
Vol 8 (3) ◽  
Author(s):  
Mandreker Bahall ◽  
Antonio Jose Reyes ◽  
Kanterpersad Ramcharan ◽  
Nadeem Hosein ◽  
Karan Seegobin ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Health Care Providers ◽  
Time Use ◽  
Idiopathic Intracranial Hypertension ◽  
Intramuscular Injection ◽  
Clinical Laboratory ◽  
Fluid Pressure ◽  
Care Providers ◽  
First Time

We report a case of a 22-year-old female student nurse who presented to hospital with an acute neuro-ophthalmological syndrome characterized by papilledema, ataxia, ophthalmoplegia and headache after a single first time use of 150 mg medroxyprogesterone intramuscular injection. Clinical, laboratory, radiological and ophthalmological investigations were in keeping with the diagnosis of idiopathic intracranial hypertension but lumbar puncture did not show a raised cerebrospinal fluid pressure suggesting a <em>forme</em> <em>fruste</em> of this entity. Her neuro-ophthalmological clinical features responded well to acetazolamide and diagnostic/ therapeutic lumbar puncture. Full recovery was achieved three months after medroxyprogesterone usage. Health care providers must be aware of this adverse drug reaction.

Predicting the need for retreatment in venous sinus stenting for idiopathic intracranial hypertension

2020 ◽  
pp. neurintsurg-2020-016550
Author(s):  
Joshua Kahan ◽  
Sri Sundararajan ◽  
Kenroy Brown ◽  
Marc Dinkin ◽  
Cristiano Oliveira ◽  
...  
Keyword(s):  
Treatment Failure ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Meta Analysis ◽  
Transverse Sinus ◽  
Venous Sinus ◽  
Unknown Etiology ◽  
Academic Center ◽  
Increased Risk

BackgroundIdiopathic intracranial hypertension is a disease of raised intra-cranial pressure of unknown etiology. Lateral cerebral venous sinus stenosis (VSS) has been increasingly reported in these patients, and stenting has emerged as an alternative treatment for medically refractory symptoms. Treatment efficacy on meta-analysis appears promising, but identifying which patients are likely to benefit most, and which are likely to require repeat procedures, is currently unclear.MethodsWe retrospectively reviewed a prospectively collected database of 79 patients treated with venous sinus stenting at a single academic center with minimum follow-up of 18 months. We extracted baseline clinical data, as well as manometry at lumbar puncture and during angiography, and used logistic regression to identify parameters that could predict stent failure.ResultsRetreatment rate after successful VSS was 13.9%. Lumbar puncture opening pressure (OP) was shown to significantly predict treatment failure (ß=0.06; OR=1.064 (1.003–1.135); P=0.039). This effect remained significant when age, sex and body mass index were added to the model (ß=0.06; OR=1.066 (1.002–1.140); P=0.043). OP was correlated with venous sinus manometry readings in the superior sagittal and transverse sinus pre-stent placement, as well trans-stenotic gradient (P<0.001).ConclusionsHigher lumbar puncture OP was associated with an increased risk of stent failure in transverse sinus stenting for idiopathic intracranial hypertension, although the performance of this model as a linear discriminator was poor. Further studies are required to better assess which patients are at greatest risk of treatment failure.

Idiopathic intracranial hypertension

Cephalalgia ◽  
2014 ◽  
Vol 35 (3) ◽  
pp. 248-261 ◽  
Author(s):  
BR Wakerley ◽  
MH Tan ◽  
EY Ting
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Treatment Guidelines ◽  
Venous Hypertension ◽  
Obese Women ◽  
Pseudotumour Cerebri ◽  
Obesity Epidemic ◽  
Fluid Production ◽  
Diagnostic Work

Background Idiopathic intracranial hypertension or pseudotumour cerebri is primarily a disorder of young obese women characterised by symptoms and signs associated with raised intracranial pressure in the absence of a space-occupying lesion or other identifiable cause. Summary The overall incidence of idiopathic intracranial hypertension is approximately two per 100,000, but is considerably higher among obese individuals and, given the global obesity epidemic, is likely to rise further. The pathophysiology of this condition is poorly understood, but most theories focus on the presence of intracranial venous hypertension and/or increased cerebrospinal fluid outflow resistance and how this relates to obesity. A lack of randomised clinical trials has resulted in unsatisfactory treatment guidelines and although weight loss is important, especially when used in conjunction with drugs that reduce cerebrospinal fluid production, resistant cases remain difficult to manage and patients invariably undergo neurosurgical shunting procedures. The use of transverse cerebral sinus stenting remains contentious and long-term benefits are yet to be determined. Conclusion An understanding of the clinical features, diagnostic work-up and therapeutic options available for patients with idiopathic intracranial hypertension is important both for neurologists and ophthalmologists as visual loss maybe permanent if untreated.

scholarly journals Infantile idiopathic intracranial hypertension: case report and review of the literature

2022 ◽  
Vol 48 (1) ◽  
Author(s):  
Francesco Del Monte ◽  
Laura Bucchino ◽  
Antonia Versace ◽  
Irene Tardivo ◽  
Emanuele Castagno ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Idiopathic Intracranial Hypertension ◽  
Symptom Relief ◽  
Clinical Manifestations ◽  
Initial Symptom ◽  
Anterior Fontanel ◽  
Opening Pressure ◽  
Cerebral Mri

Abstract Background Idiopathic intracranial hypertension is an infrequent condition of childhood, and is extremely rare in infants, with only 26 cases described. The etiology is still unknown. Typical clinical manifestations change with age, and symptoms are atypical in infants, thus the diagnosis could be late. This is based on increased opening pressure at lumbar puncture, papilloedema and normal cerebral MRI. The measurement of cerebrospinal fluid opening pressure in infants is an issue because many factors may affect it, and data about normal values are scanty. The mainstay of treatment is acetazolamide, which allows to relieve symptoms and to avoid permanent visual loss if promptly administered. Case presentation We report the case of an 8-month-old infant admitted because of vomit, loss of appetite and irritability; later, also bulging anterior fontanel was observed. Cerebral MRI and cerebrospinal fluid analysis resulted negative and after two lumbar punctures he experienced initial symptom relief. Once the diagnosis of idiopathic intracranial hypertension was made, he received oral acetazolamide, and corticosteroids, with progressive symptom resolution. Conclusions Infantile idiopathic intracranial hypertension is extremely rare, and not well described yet. Bulging anterior fontanel in otherwise healthy infants with normal neuroimaging should be always considered suggestive, but can be a late sign, while irritability and anorexia, especially if associated with vomiting, may represent an early sign. In such cases, lumbar puncture should be always done, hopefully with cerebrospinal fluid opening pressure measurement, which is among coded diagnostic criteria, but whose threshold is controversial in infants. Early diagnosis, timely treatment and strict follow-up help to prevent vision loss or death of affected infants.

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