scholarly journals Benign Intracranial Hypertension Due to Hypoparathyroidism: A Case Report

2022 ◽  
Vol 12 ◽  
Author(s):  
Giorgia Sforza ◽  
Annalisa Deodati ◽  
Romina Moavero ◽  
Laura Papetti ◽  
Ilaria Frattale ◽  
...  

Objective: The objective of this study is to present the rare case of a young girl with idiopathic intracranial hypertension secondary to hypoparathyroidism.Background: Idiopathic intracranial hypertension is a neurological syndrome characterized by elevated intracranial pressure (> 25 cmH2O) in the absence of intracerebral abnormalities or hydrocephalus. The pathophysiology of idiopathic intracranial hypertension is unknown, and rare cases of idiopathic intracranial hypertension secondary to hypoparathyroidism have been described. It is supposed that hypocalcemia causes decrease in the absorption of cerebrospinal fluid in arachnoidal granulations.Methods: The workup of the girl with idiopathic intracranial hypertension and hypoparathyroidism included physical examination, blood tests, diagnostic imaging, and lumbar puncture.Results: We present a 9-year-old female patient who was hospitalized for headache associated with nausea and vomiting for 3 weeks. She underwent an ophthalmologic examination that revealed papilledema. Lumbar puncture revealed an opening pressure of 65 cm H2O; cerebrospinal fluid analysis and brain computed tomography scan were normal. The patient started taking acetazolamide. Blood tests revealed hypocalcemia associated with high phosphorus level and undetectable PTH hormone, which led us to suspect hypoparathyroidism. She had never had cramps, paraesthesias, or tetany. Chvostek's and Trousseau's signs were positive. In the neck ultrasonography, parathyroids were not visible. Oral supplementation with calcitriol and calcium was started. Headache, nausea, and vomiting immediately disappeared after the lumbar puncture, and the papilledema improved gradually.Conclusions: Several anecdotal cases of idiopathic intracranial hypertension secondary to hypoparathyroidism have been described. However, our case report is of particular interest, since the child did not present with typical neurological hypoparathyroidism symptoms. Therefore, we recommend that hypoparathyroidism should be included in diagnostic investigations on children with clinical findings of idiopathic intracranial hypertension, because clinical manifestations of hypoparathyroidism are variable and may involve almost all organ systems.

2022 ◽  
Vol 48 (1) ◽  
Author(s):  
Francesco Del Monte ◽  
Laura Bucchino ◽  
Antonia Versace ◽  
Irene Tardivo ◽  
Emanuele Castagno ◽  
...  

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.


Cephalalgia ◽  
2020 ◽  
Vol 40 (5) ◽  
pp. 429-436 ◽  
Author(s):  
Nils J Becker ◽  
Sören Enge ◽  
Katharina Maria Kreutz ◽  
Felix Schmidt ◽  
Lutz Harms ◽  
...  

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.


2019 ◽  
Vol 34 (13) ◽  
pp. 806-814 ◽  
Author(s):  
Sama Boles ◽  
Claudia Martinez-Rios ◽  
Daniel Tibussek ◽  
Daniela Pohl

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.


2021 ◽  
Vol 12 ◽  
pp. 493
Author(s):  
Thomas Markus Dhaese ◽  
Leonardo C. Welling ◽  
Alice Magro Kosciasnki ◽  
Gustavo Frigeri ◽  
Judy Auada ◽  
...  

Background: Intracranial pressure (ICP) monitoring has been variously explored as a diagnostic and therapeutic modality in many pathological conditions leading neurological injury. This monitoring standardly depends on an invasive procedure such as cranial or lumbar catheterization. The gold standard for ICP monitoring is through an intraventricular catheter, but this invasive technique is associated with certain risks such as haemorrhage and infection. (1) Also, it is a high-cost procedure and consequently not available in a variety of underprivileged places and clinical situations in which intracranial hypertension is prevalent (3). An accurate non-invasive and low-priced method to measure elevated ICP would therefore be desirable. Under these circumstances, Brazilian scientists developed a non-invasive method for intracranial pressure monitoring (ICP-NI), which uses an electric resistance extensometer that measures micro deformations of the skull and transforms it into an electrical signal. In this case report, the authors describe a pediatrician patient with the diagnosis of idiopathic intracranial hypertension who was successfully submitted to a lumbar puncture under monitorization with this device. Case description: 7 year old girl with progressive symptoms that lead to the diagnosis of idiopathic intracranial hypertension. The patient was submitted to a lumbar punction with continuous non-invasive ICP monitoring. Conclusion: Estimating ICP (non-invasive) from LP monitoring (invasive) often reflect inaccurate ICP results, and affects negatively on IIH diagnosis and a non-invasive diagnostic method could reduce the requirement for invasive approaches, improving patient health outcomes.


Author(s):  
Manish Munjal ◽  
Monika Singla ◽  
Sahil Goel ◽  
Porshia Rishi ◽  
Nitika Tuli ◽  
...  

<p class="abstract"><span lang="EN-US">Condition of raised intracranial pressure without any mass lesion and normal cerebrospinal fluid composition is termed as idiopathic intracranial hypertension or pseudotumor cerebri. Raised intracranial tension with visual effects was treated by trans-sphenoidal optic fenestration as an emergency intervention to salvage the vision. The lamina papyracea on either side was lifted off the orbital periosteum and thick bone of the ethmoid sphenoidal junction was drilled with a diamond burr to thin it and elevate the bone covering the optic nerve.</span></p>


2019 ◽  
Vol 34 (1) ◽  
Author(s):  
Ali R. Hamdan ◽  
Amr M. Tayel ◽  
Eslam El Sayed El Khateeb ◽  
Osama Elsoghiar

Abstract Objective Assessment of visual field outcomes following the medical or surgical management of increased intracranial pressure in patients with idiopathic intracranial hypertension. Patient and methods Thirty-two patients diagnosed with idiopathic intracranial hypertension who met all the modified Dandy criteria were included in this study. Magnetic resonance brain imaging with contrast and magnetic resonance venography were performed to exclude the presence of secondary causes of intracranial hypertension. Following normal neuroimaging, a lumbar puncture was performed for all patients with papilledema to measure the cerebrospinal fluid opening pressure. The assessment of visual function, including visual acuity, fundus examination (dilated fundus examination to confirm the presence and the grade of papilledema and to exclude the other ocular causes of optic disc swelling), and formal visual field assessment by perimetry when visual acuity was more than 1/60 were performed by the ophthalmologist before and after medical or surgical treatment. Results Thirty-two patients were included, the majority of whom were females of childbearing age (29/32; 90.6%). All patients complained of headache. Visual assessment revealed the presence of blurry vision in all patients. Papilledema with different grades was observed in 31/32 (96.9%) patients, while the other patient (3.1%) showed optic atrophy; 30/32 (93.8%) patients had visual acuity ˃ 1/60. The average visual field was − 18 dB ± 9.97 SD in the right eye and − 19.47 dB ± 5.43 SD in the left eye. Twenty-nine patients received medical treatment in the form of acetazolamide, which succeeded in controlling idiopathic intracranial hypertension in 24/29 (82.7%) patients, while a theco-peritoneal shunt was inserted in 8/32 (25%) patients. After management, statistically significant improvements in cerebrospinal fluid opening pressure, presenting manifestation, visual acuity, and field were noticed. Conclusions Permanent visual loss is the most feared complication of idiopathic intracranial hypertension; therefore, early diagnosis and multidisciplinary treatment are necessary to improve visual acuity and visual field.


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