-
Journal of Neuro-ophthalmology : the... Dec 2023Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure without an identifiable cause. It mostly affects young obese women but has...
BACKGROUND
Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure without an identifiable cause. It mostly affects young obese women but has been reported in individuals newly diagnosed with anemia or with chronic anemia. The relationship between anemia and IIH is not well elucidated. This study aims to characterize the course of IIH in anemic patients.
METHODS
Consecutive patients with IIH were recruited from neuro-ophthalmology clinics. Subsequent retrospective chart review obtained information on symptoms, complete blood counts (CBCs), visual acuity, visual fields, and optic disc edema at baseline and follow-up. Anemia was diagnosed by hemoglobin <120 g/L (women) and <130 g/L (men).
RESULTS
One hundred twenty-three patients with IIH were recruited for this study, and 22.8% (28/123) had anemia. More anemic individuals had mild-to-moderate visual acuity impairment (logarithm of minimum angle of resolution 0.3-1, P = 0.01) and worse automated mean deviation ( P = 0.048). The median follow-up time was 47.4 weeks (interquartile range:20.1-91.8). Fifteen of 28 patients with anemia received anemia treatment and showed a trend toward increased hemoglobin. At follow-up, there were no differences in the visual acuity, but patients with anemia had worse automated mean deviation on visual fields ( P = 0.045).
CONCLUSIONS
IIH patients with anemia had worse visual function at presentation and worse final visual field parameters. This suggests that CBCs should be obtained for patients with papilledema because it may influence final visual outcome.
Topics: Male; Humans; Female; Pseudotumor Cerebri; Retrospective Studies; Intracranial Hypertension; Papilledema; Anemia; Vision, Low; Hemoglobins
PubMed: 36255106
DOI: 10.1097/WNO.0000000000001715 -
Journal of the Neurological Sciences Sep 2023Fulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous...
BACKGROUND
Fulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous stenting (CTVS) is efficacious in IIH patients, but emergent CTVS in FIIH is rarely reported. We present our experience with emergent CTVS in patients with FIIH.
METHODS
Since 01/2019, an institutional protocol allowed emergent CTVS in FIIH patients with bilateral transverse sinus stenosis and gradient pressure > 15 on digital subtraction angiography (DSA). We retrospectively analyzed a prospective registry of all IIH patients with details of neurological and neuro-ophthalmological assessments before and after treatment, and subjective assessments of headache and tinnitus were made pre-and post-procedure.
RESULTS
259 IIH patients, including 49 who underwent CTVS, were registered. Among them, five female patients met inclusion criteria for FIIH and underwent emergent CTVS. FIIH patients were younger (18.8 ± 1.64 vs 27.7 ± 4.85, p < 0.01), mean BMI was lower (30.8 ± 10.57 vs 34.6 ± 4.3, p < 0.01), and lumbar puncture opening pressure higher (454 ± vs 361 ± 99.4, p < 0.01) than that of IIH patients. They presented with acute visual loss, severe headache, papilledema, significant bilateral transverse sinus stenosis on CT-venography, and mean dominant side gradient pressure of 26.4 ± 6.2 on DSA. CTVS was performed without significant complications, resulting in remarkable improvement in headache, optical coherence tomography, and visual fields within 1 week. At 1-year follow-up (four patients) and 6-month follow-up (1 patient), there was complete resolution of papilledema and headache, and marked improvement in visual acuity.
CONCLUSIONS
In these patients, emergent-CTVS was a safe and effective treatment option for FIIH. Further evaluation is warranted.
Topics: Humans; Female; Pseudotumor Cerebri; Papilledema; Retrospective Studies; Constriction, Pathologic; Headache; Stents; Vision Disorders; Cranial Sinuses; Intracranial Hypertension
PubMed: 37572407
DOI: 10.1016/j.jns.2023.120761 -
Journal of Women's Health (2002) Jan 2024
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension
PubMed: 37713299
DOI: 10.1089/jwh.2023.0373 -
Acta Radiologica (Stockholm, Sweden :... Jun 2024The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several...
BACKGROUND
The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several imaging markers have been identified as potential predictors of IIH.
PURPOSE
To investigate the predictive roles of novel radiological markers as the Meckel's cave area, alongside classical radiologic markers in identifying IIH such as the empty sella.
MATERIAL AND METHODS
The patients were classified according to cerebrospinal fluid (CSF) opening pressure as the IIH group and control group. The observational, case-control study included 22 patients with IIH and 22 controls. Groups were compared for presence of empty sella, Meckel's cave area, fat area of posterior neck, fat thickness of scalp, presence of transverse sinus stenosis, and ophthalmic markers, such as increase of optic nerve (ON) sheath diameter.
RESULTS
In the IHH group, higher occurrences of increased ON sheath diameter, ON tortuosity, flattening of the scleral surface, and transverse sinus stenosis were observed ( < 0.001, < 0.001, = 0.046, and = 0.021, respectively). Meckel's cave area and fat area of posterior neck were similar in both groups ( = 0.444 and = 0.794).
CONCLUSION
Ophthalmic markers and transverse sinus stenosis could be utilized as radiologic features supporting early and precise diagnosis of IIH. However, enlargement of Meckel's cave area and measurements of fatty area of posterior neck are not helpful for diagnosis of IIH.
PubMed: 38870347
DOI: 10.1177/02841851241256008 -
Stenting versus medical treatment for idiopathic intracranial hypertension: a matched-control study.Journal of Neurointerventional Surgery Oct 2023This prospective cohort study compared the outcomes of stenting and medical treatment for patients with idiopathic intracranial hypertension (IIH) and venous sinus...
BACKGROUND
This prospective cohort study compared the outcomes of stenting and medical treatment for patients with idiopathic intracranial hypertension (IIH) and venous sinus stenosis (VSS).
METHODS
In this single-center cohort study, patients with IIH and VSS were evaluated between January 2014 and December 2019 with follow-up periods of 1, 3, and 6 months. The patients received either stenting or medical treatment. The two groups underwent 1:1 matching using propensity score analysis, and the clinical outcomes were compared.
RESULTS
Following 1:1 matching, 36 patients who underwent stenting and 36 who underwent medical treatment were matched. The median improvements in the papilledema Frisén grade were greater in the stenting group at 1 month (-2 vs 0), 3 months (-3 vs -1), and 6 months (-3 vs -1) than in the medical treatment group. Patients who received stenting treatment had a significantly higher prevalence of complete resolution of their respective symptoms (headache, tinnitus, or visual disturbances) at 3 months (58.3% vs 13.9%, OR 8.68, 95% CI 2.74 to 27.52) and 6 months (80.6% vs 22.2%, OR 14.50, 95% CI 4.64 to 45.32) than those receiving medical treatment.
CONCLUSIONS
This matched-control study shows that stenting has a greater efficacy rate and rapid resolution of papilledema and its respective symptoms compared with medical treatment.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Cohort Studies; Prospective Studies; Treatment Outcome; Stents; Constriction, Pathologic; Cranial Sinuses; Intracranial Hypertension; Retrospective Studies
PubMed: 36202600
DOI: 10.1136/jnis-2022-019191 -
Ophthalmology Science 2024To develop and test an artificial intelligence (AI) model to aid in differentiating pediatric pseudopapilledema from true papilledema on fundus photographs.
PURPOSE
To develop and test an artificial intelligence (AI) model to aid in differentiating pediatric pseudopapilledema from true papilledema on fundus photographs.
DESIGN
Multicenter retrospective study.
SUBJECTS
A total of 851 fundus photographs from 235 children (age < 18 years) with pseudopapilledema and true papilledema.
METHODS
Four pediatric neuro-ophthalmologists at 4 different institutions contributed fundus photographs of children with confirmed diagnoses of papilledema or pseudopapilledema. An AI model to classify fundus photographs as papilledema or pseudopapilledema was developed using a DenseNet backbone and a tribranch convolutional neural network. We performed 10-fold cross-validation and separately analyzed an external test set. The AI model's performance was compared with 2 masked human expert pediatric neuro-ophthalmologists, who performed the same classification task.
MAIN OUTCOME MEASURES
Accuracy, sensitivity, and specificity of the AI model compared with human experts.
RESULTS
The area under receiver operating curve of the AI model was 0.77 for the cross-validation set and 0.81 for the external test set. The accuracy of the AI model was 70.0% for the cross-validation set and 73.9% for the external test set. The sensitivity of the AI model was 73.4% for the cross-validation set and 90.4% for the external test set. The AI model's accuracy was significantly higher than human experts on the cross validation set ( < 0.002), and the model's sensitivity was significantly higher on the external test set ( = 0.0002). The specificity of the AI model and human experts was similar (56.4%-67.3%). Moreover, the AI model was significantly more sensitive at detecting mild papilledema than human experts, whereas AI and humans performed similarly on photographs of moderate-to-severe papilledema. On review of the external test set, only 1 child (with nearly resolved pseudotumor cerebri) had both eyes with papilledema incorrectly classified as pseudopapilledema.
CONCLUSIONS
When classifying fundus photographs of pediatric papilledema and pseudopapilledema, our AI model achieved > 90% sensitivity at detecting papilledema, superior to human experts. Due to the high sensitivity and low false negative rate, AI may be useful to triage children with suspected papilledema requiring work-up to evaluate for serious underlying neurologic conditions.
FINANCIAL DISCLOSURES
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
PubMed: 38682028
DOI: 10.1016/j.xops.2024.100496 -
Neuro-ophthalmology (Aeolus Press) 2024Nitrous oxide is used as an anaesthetic and analgesic agent in the medical setting and is known to cause raised intracranial pressure. The use of nitrous oxide...
Nitrous oxide is used as an anaesthetic and analgesic agent in the medical setting and is known to cause raised intracranial pressure. The use of nitrous oxide recreationally for the drug's euphoric and relaxant properties has been linked to multiple neurological and psychiatric sequelae including neuropathy, myelopathy, and psychosis. We describe a case of a young person who declared heavy nitrous oxide use resulting in vision-threatening papilloedema secondary to raised intracranial pressure. He underwent emergency lumbar drainage alongside high-dose acetazolamide and parenteral vitamin B injections. To our knowledge, there have yet to be other reports of cases where heavy nitrous oxide use has caused secondary pseudotumor cerebri syndrome.
PubMed: 38933749
DOI: 10.1080/01658107.2023.2301359 -
Obesity Surgery Aug 2023Idiopathic Intracranial Hypertension (IIH) is a rare disorder, linked to severe obesity. The study aimed to evaluate long-term effects of metabolic and bariatric surgery...
BACKGROUND
Idiopathic Intracranial Hypertension (IIH) is a rare disorder, linked to severe obesity. The study aimed to evaluate long-term effects of metabolic and bariatric surgery (MBS) on IIH outcomes.
METHODS
Retrospective study of patients with IIH and severe obesity who underwent MBS. Data were retrieved from prospectively maintained databases of two bariatric surgeons.
RESULTS
Thirteen patients were included, of them 12 women. Median age was 36 (interquartile range;IQR 21,47) years and body mass index (BMI) was 40.4 (IQR 37.8,41.8) kg/m2. All patients had visual disturbances,12/13 had headaches, and 6/13 had tinnitus. The mean opening pressure on lumbar puncture was 45 cmH2O, and 11/13 patients had papilledema. Medications for IIH were consumed by 11/13 patients, and 2/13 patients had prior surgical intervention for IIH. MBS types included sleeve gastrectomy (n=7), adjustable gastric banding (n=2), roux-en-y gastric bypass (n=2), one anastomosis gastric bypass (n=1), duodenal switch (n=1). At a median follow-up of 10 years (IQR 8,13), the median BMI and total weight loss were 29.7 kg/m2 and 27%, respectively. Remission of symptoms was achieved in 9/13 patients.
CONCLUSIONS
MBS results in significant and sustainable weight loss, with subsequent resolution or improvement of IIH. It may be considered as a preventive measure for IIH in patients with severe obesity.
Topics: Humans; Female; Adult; Obesity, Morbid; Pseudotumor Cerebri; Retrospective Studies; Bariatric Surgery; Obesity; Gastric Bypass; Weight Loss; Gastrectomy; Treatment Outcome
PubMed: 37351765
DOI: 10.1007/s11695-023-06696-y -
CEN Case Reports Nov 2023TRPM6 is predominantly expressed in the kidney and colon and encodes a protein containing an ion channel domain and a protein kinase domain. It is crucial for magnesium... (Review)
Review
TRPM6 is predominantly expressed in the kidney and colon and encodes a protein containing an ion channel domain and a protein kinase domain. It is crucial for magnesium homeostasis and plays important roles in epithelial magnesium transport and the active magnesium absorption. In this study, we present a 70-day-old Iranian female patient from consanguineous parents with hypomagnesemia and secondary hypocalcemia. She presented with seizures 19 days after birth and refractory watery non-bloody diarrhea. She consequently had failure to thrive. Other features included hypotonia, wide anterior fontanel, ventriculomegaly, and pseudotumor cerebri following administration of nalidixic acid. She had severe hypomagnesemia and hypocalcemia which were treated with magnesium and calcium supplementation. Despite initial unstable response to supplemental magnesium, she eventually improved and the diarrhea discontinued. The patient was discharged by magnesium and calcium therapy. At the last follow-up at age 2.5 years, the patient remained well without any recurrence or complication. Genetic testing by whole-exome sequencing revealed a novel homozygous frameshift insertion-deletion (indel) variant in exon 26 of the TRPM6 gene, c.3693-3699del GCAAGAG ins CTGCTGTTGACATCTGCT, p.L1231Ffs*36. Segregation analysis revealed the TRPM6 heterozygous variant in both parents. Patients with biallelic TRPM6 pathogenic variants typically exhibit hypomagnesemia with secondary hypocalcemia and present with neurologic manifestations including seizures. In some patients, this is also complicated by chronic diarrhea and failure to thrive. Long-term complications are rare and most of the patients show a good prognosis with supplemental magnesium therapy.
Topics: Female; Humans; Calcium; Diarrhea; Failure to Thrive; Hypocalcemia; Iran; Magnesium; Seizures; TRPM Cation Channels; Aged
PubMed: 36967423
DOI: 10.1007/s13730-023-00785-8 -
Interventional Neuroradiology : Journal... Aug 2023Venous sinus stenosis (VSS) stenting has emerged as an effective treatment for patients with Idiopathic Intracranial Hypertension (IIH). However, stenting carries risk...
BACKGROUND
Venous sinus stenosis (VSS) stenting has emerged as an effective treatment for patients with Idiopathic Intracranial Hypertension (IIH). However, stenting carries risk of in-stent stenosis/thrombosis and cumulative bleeding risk from long-term dual antiplatelet (DAPT) use. Thus, we investigated the potential safety and efficacy of primary balloon angioplasty as an alternative to stenting in IIH.
METHODS
A prospectively maintained single-center registry of IIH patients undergoing endovascular procedures was queried. Inclusion criteria included patients with confirmed IIH and angiographically demonstrable VSS who underwent interventions from 2012- 2021. Patients were dichotomized into primary balloon angioplasty (Group A) and primary stenting (Group S), comparing clinical outcomes using bivariate analyses.
RESULTS
62 patients were included with median age of 33 [IQR 26-37], 74% females. Group A (9/62) and Group S (53/62) had similar baseline characteristics. Papilledema improvement was higher in Group S at 6 weeks and 6 months (44 vs. 93, p = 0.002 and 44 vs. 92%, p = 0.004), with similar improvements across all symptoms. Group S had higher mean post-procedure venous pressure gradient change (8 vs. 3 mmHg, p = 0.02) and a lower CSF opening pressure at 6 months (23 vs. 36 cmH2O, p < 0.001). VPS rescue rate was higher in Group A (44 vs. 2%, p = 0.001). There was only one procedural complications; a subdural hematoma in Group A.
CONCLUSIONS
Primary VSS balloon angioplasty provides a marginal and short-lived improvement of IIH symptoms compared to stenting. These findings suggest a cautious and limited role for short-term rescue angioplasty in poor shunting and stenting candidates with refractory IIH.
Topics: Female; Humans; Male; Pseudotumor Cerebri; Constriction, Pathologic; Cranial Sinuses; Treatment Outcome; Angioplasty, Balloon; Stents; Intracranial Hypertension; Retrospective Studies
PubMed: 35323053
DOI: 10.1177/15910199221089446