Rapidly progressive vision loss due to fulminant idiopathic intracranial hypertension: a diagnostic and management dilemma

2020 ◽  
Vol 13 (11) ◽  
pp. e236188
Author(s):  
Jagadeesh Sutraye ◽  
Mohan Kannam ◽  
Rajat Kapoor ◽  
Virender Sachdeva
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Obese Woman ◽  
Visual Fields ◽  
Csf Analysis ◽  
Contrast Enhanced ◽  
Grade Ii ◽  
The Right ◽  
The Brain

A 44-year-old obese woman presented with decrease in vision in the right eye (RE) for 3 days. She reported a simultaneous onset of holocranial headache that worsened on bending forward. She denied eye pain, pain on eye movements, and other ocular or neurological complaints. On examination, her distance best-corrected visual acuity was counting fingers at 1 m in the RE and 20/20 in the left eye (LE). Colour vision was subnormal in both eyes (BE). There was grade II relative afferent pupillary defect in the RE. Fundus examination showed disc oedema in BE . Visual fields in the LE showed central scotoma extending nasally. A provisional diagnosis of papillitis was considered. However, contrast-enhanced MRI of the brain and orbits showed evidence of elevated intracranial pressure. Cerebrospinal fluid (CSF) opening pressure was 42 cm H2O while rest of the CSF analysis was normal. Diagnosis was revised to fulminant idiopathic intracranial hypertension. Management with medical therapy and urgent thecoperiteoneal shunt improved visual function in BE.

ASSESSMENT OF PHYSIOLOGICAL PARAMETERS OF THE TRIGEMINAL AND COCHLEOVESTIBULAR SYSTEMS IN TEMPOROMANDIBULAR JOINT PAIN DYSFUNCTION

2020 ◽  
Vol 17 (2) ◽  
pp. 110-120
Author(s):  
N.D. Sorokina ◽  
◽  
L.R. Shahalieva ◽  
S.S. Pertsov ◽  
L.V. Polma ◽  
...  
Keyword(s):  
Nervous System ◽  
Evoked Potentials ◽  
Temporomandibular Joint ◽  
Differential Diagnostics ◽  
Autonomous Nervous System ◽  
Dental Diseases ◽  
Grade Ii ◽  
The Right ◽  
Distal Occlusion ◽  
The Brain

One of the most common causes of chronic pain in the facial region, including in the trigeminal nerve link, which is not associated with dental diseases, is pain dysfunction of the temporomandibular joint. At the same time, there is evidence in the literature that there are relationships between pain dysfunction of the temporomandibular joint, abnormal occlusion, cervical-muscular tonic phenomena, postural disorders, dysfunction of the Autonomous nervous system and cochleovestibular manifestations. At the same time, neurophysiological indicators of functional disorders in the maxillofacial region and intersystem interactions in pain dysfunction of the temporomandibular joint are insufficiently studied.Goal. The aim of the work is to evaluate the neurophysiological features of trigeminal afferentation in terms of trigeminal somatosensory evoked potentials (TSWP) and the auditory conducting system of the brain in terms of acoustic stem evoked potentials (ASVP) in distal occlusion of the dentition with pain dysfunction of the temporomandibular joint (TMJ) in comparison with physiological occlusion in students 18-21 years old. Material and methods. The main study included 41 students with distal occlusion (21 girls and 20 boys), (grade II Engl, symmetrically right and left in 14 people, and grade II Engl on the left and grade I on the right in 12 people, grade I on the left and grade II on the right in 15 people). All respondents with distal occlusion and who were practically healthy signed an informed consent to participate in the study. We used complex orthodontic methods of examination, subjective degree of severity and intensity of pain in the TMJ, assessment of the Autonomous nervous system (samples and tests), and neurophysiological methods for assessing TSVP and ASVP. Results. Significant differences in ASEP parameters were found in the group of respondents with distal occlusion in the form of a decrease in the latency period of peak I, III, and V compared to physiological occlusion, that correlated with the subjective assessment (in points) of cochleovestibular disorders. According to the TSVP study, a decrease in the duration of latent periods was found, which indicates an increased excitability of non-specific brain stem structures at the medullo-ponto-mesencephalic level compared to the control group. Conclusions. The results obtained are supposed to be used for differential diagnostics, including such dental diseases as TMJ pain dysfunction, occlusion abnormalities accompanied by pain syndrome. Additional functional diagnostics of multi-modal VP of the brain (acoustic evoked potentials, trigeminal evoked potentials) can be performed in conjunction with indicators of autonomic nervous system dysfunction, with parameters of severity of clinical symptoms of cochleovestibular disorders, musculoskeletal dysfunction the maxillofacial area, with indicators of pain, which will determine the tactics and effectiveness of subsequent treatment.

scholarly journals Intracranial Venous Sinus Stenting in Idiopathic Intracranial Hypertension: A Case Report and Review of the Literature

2021 ◽  
Vol 11 (3) ◽  
pp. 382
Author(s):  
Dinesh Ramanathan ◽  
Zachary D. Travis ◽  
Emmanuel Omosor ◽  
Taylor Wilson ◽  
Nikhil Sahasrabudhe ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Transverse Sinus ◽  
Venous Sinus ◽  
Subdural Hygroma ◽  
Presenting Symptoms ◽  
Minimal Improvement ◽  
Sinus Stenosis ◽  
Work Up

We describe a case of severe headaches, double vision, and progressive vision loss secondary to a ruptured intracranial cyst (IAC) in a 31-year-old woman with no relevant past medical history. The case is peculiar because drainage of the subdural hygroma led to a minimal improvement in vision with persistent elevated intracranial pressure (ICP). Further exploration revealed transverse sinus stenosis necessitating stenting. Evaluation post-stenting showed marked reduction of ICP and improvement in symptoms. This report underscores the importance of comprehensive work-up and suspicion of multiple underlying etiologies that may be crucial to complete resolution of presenting symptoms in some cases. We provide an overview of the clinical indications and evidence for venous sinus stenting in treating idiopathic intracranial hypertension (IIH).

Abstract 1122‐000232: The Importance of Performing Venous Pressure Measurements Following Venous Sinus Stenting for Idiopathic Intracranial Hypertension

2021 ◽  
Vol 1 (S1) ◽  
Author(s):  
Yazan Radaideh
Keyword(s):  
Pressure Gradient ◽  
Intracranial Hypertension ◽  
Stent Placement ◽  
Idiopathic Intracranial Hypertension ◽  
Venous Pressure ◽  
Sigmoid Sinus ◽  
Venous Sinus ◽  
Pressure Measurements ◽  
Sinus Stenosis ◽  
The Right

Introduction : Although venous sinus stenting is an established treatment for medically refractory idiopathic intracranial hypertension, a subset of patients shows little or no improvement of symptoms after stenting. While this could be related to a number of factors, failure to sufficiently address the pressure gradient is one that can be recognized during the treatment procedure. We describe two patients who had a persistent venous pressure gradient after stent placement. Once identified, a second stent was placed with subsequent resolution of the pressure gradient. Methods : This retrospective chart review identified patients at a single institution who underwent venous sinus stenting and required immediate placement of a second venous sinus stent for a persistent pressure gradient. Results : Two patients with refractory idiopathic intracranial hypertension underwent cerebral angiography with venous manometry. In the first patient, unilateral venous sinus stenosis was present with a maximum pressure of 50 mmHg, which only decreased to 30 mmHg after placement of a right transverse‐sigmoid sinus stent. Subsequent manometry revealed a persistent gradient between the superior sagittal sinus and the right transverse sinus, which resolved after placement of a second stent in this location. In the second patient, bilateral stenosis was observed at the transverse‐sigmoid sinus junction; the maximum venous pressure was 40 mmHg, and a gradient of 30 mmHg was measured at the right transverse‐sigmoid junction, where a venous sinus stent was placed. Venous sinus pressure measurements performed immediately after the stent placement demonstrated a persistent pressure gradient of 20 mmHg in the contralateral transverse‐sigmoid sinus junction, which resolved after contralateral stent placement. Both patients showed sustained improvement in their symptoms at 1 year follow up. Conclusions : In some patients with idiopathic intracranial hypertension and venous sinus stenosis, a single stent may not sufficiently reduce the pressure gradient. A second stent may be required; however, this is only detectable with post‐stent pressure measurements. Performing manometry after stent placement should be routinely performed in order to detect persistent venous pressure gradient.

scholarly journals A Case of Pulsatile Tinnitus Related to Idiopathic Intracranial Hypertension

2020 ◽  
Vol 63 (9) ◽  
pp. 422-426
Author(s):  
Jong-Won Bae ◽  
Kyu-Yup Lee ◽  
Myung Hoon Yoo ◽  
Da Jung Jung
Keyword(s):  
Intracranial Hypertension ◽  
Young Women ◽  
Idiopathic Intracranial Hypertension ◽  
Obese Woman ◽  
Pulsatile Tinnitus ◽  
Obese Women ◽  
Women Of Childbearing Age ◽  
Childbearing Age ◽  
Early Symptom ◽  
Vascular Abnormalities

Idiopathic intracranial hypertension (IIH) is a disorder that commonly occurs in obese young women of childbearing age and is characterized by symptoms such as pulsatile tinnitus, dizziness, headache, nausea, vomiting, and visual loss without any structural or vascular abnormalities in the intracranial cavity. We recently experienced a case of a 33-year-old obese woman who presented with right-sided pulsatile tinnitus, which is an early symptom for IIH. The patient was successfully treated with weight reduction and carbonic anhydrase inhibitor (acetazolamide). Pulsatile tinnitus requires thorough diagnosis and examination because it can be cured if the anatomical or functional cause is identified and treated. When obese women of childbearing age present with pulsatile tinnitus at the otorhinolaryngology outpatient department, treatment for IIH should be initiated after appropriate examination and diagnosis as pulsatile tinnitus may be the only symptom for IIH.

Idiopathic intracranial hypertension

Neurology ◽  
2018 ◽  
Vol 91 (11) ◽  
pp. 515-522 ◽  
Author(s):  
Stéphanie Lenck ◽  
Ivan Radovanovic ◽  
Patrick Nicholson ◽  
Mojgan Hodaie ◽  
Timo Krings ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Interstitial Fluid ◽  
Water Exchange ◽  
Venous Blood ◽  
Glymphatic System ◽  
Associated Conditions ◽  
Outflow Pathway ◽  
The Brain

The recent discoveries of the glymphatic and lymphatic systems of the brain have helped advance our understanding of CSF physiology and may allow new insights in the understanding of idiopathic intracranial hypertension (IIH). The clinical and radiologic presentations of IIH appear to be related to congestion of the glymphatic system associated with an overflow of the lymphatic CSF outflow pathway. By revisiting the role of “vascular arachnoid granulations” in the brain, we hypothesize that an initial impairment of the transport of interstitial fluid from the glymphatic system to the venous blood of the dural sinuses may trigger the hydrodynamic cascade of IIH. Furthermore, we speculate that, similar to other water-exchange systems in the brain, a specific subtype of aquaporin is involved in this transport. This theory may eventually help to provide an underlying explanation for IIH and its associated conditions, since in most of them, the expression of several aquaporins is altered.

External lumbar drainage in progressive pediatric idiopathic intracranial hypertension

2021 ◽  
pp. 1-7
Author(s):  
Gad Dotan ◽  
Natalie Hadar Cohen ◽  
Hanya M. Qureshi ◽  
Mika Shapira Rootman ◽  
Yoram Nevo ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Symptom Relief ◽  
Visual Outcome ◽  
Fiber Layer ◽  
Lumbar Drainage ◽  
Lumbar Drain ◽  
Rnfl Thickness ◽  
External Lumbar Drainage

OBJECTIVE Pediatric idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure despite normal cerebrospinal fluid and neuroimaging findings. Initial management is typically medical; however, nearly 10% of children will eventually require surgery for persistent headache and/or vision loss. External lumbar drainage, which is a considerably safer treatment option, has not been adequately analyzed in children with medically refractory IIH. METHODS The authors conducted a single-institution retrospective analysis of children with medically refractory IIH who had undergone external lumbar drain (ELD) placement because of worsening papilledema, reflected as increased retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) testing. The main outcome measures were effects of external lumbar drainage on papilledema resolution, symptoms, and vision. RESULTS The authors analyzed the medical records of 13 children with IIH (11 girls, mean age 15.0 ± 2.3 years) whose mean CSF opening pressure was 45.5 ± 6.8 cm H2O. In all children, the average global RNFL thickness in both eyes significantly increased at ELD placement (right eye 371.8 ± 150.2 μm, left eye 400.3 ± 96.9 μm) compared with presentation thickness (right eye 301.6 ± 110.40 μm, left eye 350.2 ± 107.7 μm) despite acetazolamide medical therapy (20–30 mg/kg/day), leading to ELD placement after 9.5 ± 6.9 days (range 3–29 days). After ELD insertion, there was headache resolution, gradual and continuous improvement in optic disc thickness, and preservation of good vision. CONCLUSIONS ELD placement in children with medically refractory IIH who demonstrated worsening papilledema with increased RNFL thickening on OCT testing typically results in symptom relief and disc edema resolution with good visual outcome, often preventing the need for additional definitive surgeries that carry greater failure and morbidity risks.

Treatment of idiopathic intracranial hypertension via stereotactic placement of biventriculoperitoneal shunts

2018 ◽  
Vol 130 (1) ◽  
pp. 136-144 ◽  
Author(s):  
Michael Karsy ◽  
Hussam Abou-Al-Shaar ◽  
Christian A. Bowers ◽  
Richard H. Schmidt
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Single Case ◽  
Distal Catheter ◽  
Shunt Placement ◽  
Presenting Symptoms ◽  
Small Series ◽  
Shunt Survival

OBJECTIVEIdiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a complex and difficult-to-manage condition that can lead to permanent vision loss and refractory headaches if untreated. Traditional treatment options, such as unilateral ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt placement, have high complication and failure rates and often require multiple revisions. The use of bilateral proximal catheters has been hypothesized as a method to improve shunt survival. The use of stereotactic technology has improved the accuracy of catheter placement and may improve treatment of IIH, with fewer complications and greater shunt patency time.METHODSThe authors performed a retrospective chart review for all patients with IIH who underwent stereotactic placement of biventriculoperitoneal (BVP) shunt catheters from 2008 to 2016 at their institution. Bilateral proximal catheters were Y-connected to a Strata valve with a single distal catheter. We evaluated clinical, surgical, and ophthalmological variables and outcomes.RESULTSMost patients in this series of 34 patients (mean age 34.4 ± 8.2 years, mean body mass index 38.7 ± 8.3 kg/m2; 91.2% were women) undergoing 41 shunt procedures presented with headache (94.1%) and visual deficits (85.3%). The mean opening pressure was 39.6 ± 9.0 cm H2O. In addition, 50.0% had undergone previous unilateral shunt placement, and 20.6% had undergone prior optic nerve sheath fenestration. After BVP shunt placement, there were no cases of proximal catheter obstruction and only a single case of valve obstruction at 41.9 months, with a mean follow-up of 24.8 ± 20.0 months. Most patients showed improvement in their headache (82.4%), subjective vision (70.6%), and papilledema (61.5% preoperatively vs 20.0% postoperatively, p = 0.02) at follow-up. Additional primary complications included 4 patients with migration of their distal catheters out of the peritoneum (twice in 1 patient), and an infection of the distal catheter after catheter dislodgment. The proximal obstructive shunt complication rate in this series (2.9%) was lower than that with LP (53.5%) or unilateral VP (37.8%) shunts seen in the literature.CONCLUSIONSThis small series suggests that stereotactic placement of BVP shunt catheters appears to improve shunt survival rates and presenting symptoms in patients with IIH. Compared with unilateral VP or LP shunts, the use of BVP shunts may be a more effective and more functionally sustained method for the treatment of IIH.

scholarly journals Vietnamese patient with progressive ataxia and palatal tremor syndrome

2015 ◽  
Vol 42 (S1) ◽  
pp. S31-S31
Author(s):  
A Jahangirvand ◽  
K Sawicka ◽  
S Kennedy
Keyword(s):  
Inferior Olive ◽  
Facial Weakness ◽  
Palatal Tremor ◽  
Mri Features ◽  
Unsteady Gait ◽  
Contrast Enhanced ◽  
Progressive Ataxia ◽  
The Right ◽  
The Brain ◽  
And Diffusion

Background: We present clinical and MRI features of progressive ataxia and palatal tremor (PAPT). Case Report: A 67 year-old gentleman visiting from Vietnam presented with intermittent stroke-like episodes consisting of facial weakness, dysarthria, and osillopsia. He reported gradually worsening ataxia and dysequilibrium over 4 years. Examination revealed small amplitude nystagmus towards the right, impaired VOR to the left, palatal tremor, left-sided dysmetria, and an unsteady gait. MRI of the brain demonstrated increased T2/FLAIR signal within the inferior olive. Contrast enhanced and diffusion sequences were normal. MRA was normal. Electrocardiography, telemetry, and echocardiogram were normal. CSF was normal. Glial fibrillary acidic protein (GFAP) and vitamin E levels were normal. Genetic testing for hereditary forms of ataxia including spinocerebellar ataxia was not completed. Conclusion: Palatal tremor is commonly classified into symptomatic or essential subgroups. Symptomatic palatal tremor is frequently caused by a lesion in the triangle of Guillain and Mollaret leading to hypertrophic olivary degeneration. A subgroup of symptomatic palatal tremor form a syndrome of PAPT. Published details of cases of PAPT are sparse and the disorder appears to be mainly sporadic. Common features include progression of ataxia, olivary degeneration, gaze-evoked nystagmus, and internuclear ophthalmoplegia. There is no known effective treatment for progressive ataxia, which is the most disabling symptom of PAPT.

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