Alternative Path for Optic Nerve Decompression in Pseudotumor Cerebri With Full Endoscopic Lateral Transorbital Approach.

Endonasal endoscopic approaches are the most preferred surgical methods in patients with pseudotumor cerebri because of easy access to the optic nerve, but the choice of this technique may not apply to all endoscopic endonasal cases. Moreover, there may be difficulties in practice in some cases, including the coronavirus disease 2019 pandemic. This study aimed to suggest an alternative endoscopic approach by lateral orbitotomy for optic nerve decompression in patients with pseudotumor cerebri. The study was performed using 5 fresh-frozen cadaver heads (bilaterally, total of 10 sides) injected intravenously with colored silicone preserved in the cold chain. An average of 2.5 cm skin incision was made to fit the lateral orbitotomy. The lengths of the recurrent meningeal artery (mm), the meningo-orbital band (mm), and the optic nerve (mm) to the orbital margin were measured. After these morphometric measurements, optic nerve decompression was performed endoscopically, and the length of the decompression was measured (mm). The average length (mm) between the orbital rim and meningeal recurrent artery (or meningolacrimal branch) was 16.2 mm, between the orbital rim and the meningo-orbital band was 18.5 mm, and between the orbital rim and optic nerve was 44.1 mm. The average optic nerve decompression length was 4.4 mm. The endoscopic lateral orbitotomy approach provides easy access to the optic nerve by anatomically following the recurrent meningeal artery and the meningo-orbital band. It can be a safe second-line approach after endonasal approaches for optic nerve decompression in pseudotumor cerebri.

Headache, Loss of Smell, and Visual Disturbances: Symptoms of SARS-CoV-2 Infection? A Case Report.

OBJECTIVE: The purpose of this case report is to describe the main components of the history and physical examination that led to idiopathic intracranial hypertension differential diagnosis, which initially was investigated as COVID-19. METHODS (CASE DESCRIPTION): A 28-year-old woman complaining of constant headache and loss of smell and taste was suspected as SARS-CoV-2 infection by her general practitioner. She underwent 3 molecular swab tests, all negative, then decided to seek her physical therapist for relieving headache. RESULTS: The full cranial nerve examination revealed impaired olfactory (CNI), abducens (CN VI), and facial (CN VII) nerves, leading the physical therapist to refer the patient to a neurosurgeon for a suspected central nervous system involvement. The neurosurgeon prescribed a detailed MRI and an ophthalmologic examination, which allowed for the final diagnosis of idiopathic intracranial hypertension. CONCLUSION: An urgent lumbo-peritoneal shunting surgery resolved the patient's symptoms and saved her sight. Despite the ongoing COVID-19 pandemic, health care professionals must pay attention to properly investigating patients' signs and symptoms using comprehensive clinical reasoning, considering the screening for referral to specialist medical attention. IMPACT: A thorough physical examination is required for every patient even if patients' signs and symptoms are in line with apparent common and widespread pathologies. Cranial nerve evaluation is an essential component of the physical therapist assessment and decision-making process. The ongoing pandemic highlighted the fundamental assistance of physical therapists toward physicians in the screening and management of musculoskeletal diseases.

Metabolic acidosis during the relapse treatment of idiopathic intracranial hypertension.

We herein present a 40-year-old female physician who was diagnosed with idiopathic intracranial hypertension (IIH) 4 years ago. In the last years, the patient was in remission without any medications. Since the onset of COVID-19 pandemic, she has been stressfully working in the high-risk area, therefore using personal protective equipment (N95 mask, protective clothing, goggles, and protective cap) during the day for extended periods. Her headaches recurred and the patient was diagnosed with a relapse of IIH; acetazolamide and afterward topiramate were initiated, with diet treatment. Symptomatic metabolic acidosis, which is otherwise a rare side effect of the IIH treatment and not seen in her first attack even with higher doses, developed during the follow-up, presenting with shortness of breath and chest tightening. The emerging problems of IIH diagnosis and management during the COVID-19 pandemic will be discussed.

[Impact of COVID-19 lockdown on patients with idiopathic intracranial hypertension in Spain]. / Impacto del confinamiento por COVID-19 en pacientes con hipertensión intracraneal idiopática en España.

INTRODUCTION: The nationwide lockdown implemented in Spain in March 2020 in response to the 2019 coronavirus disease pandemic (COVID-19) could have had an effect on the clinical situation, disease management and access to care in patients with idiopathic intracranial hypertension. PATIENTS AND METHODS: A cross-sectional observational study was conducted by means of an online survey. The frequency and impact of headache, visual symptoms, dizziness, cognitive symptoms, diplopia, anxiety and depression on patients' quality of life were recorded. Disease treatment and delays experienced in accessing care were recorded. Participants were grouped according to the time elapsed since diagnosis. RESULTS: A total of 112 patients participated in the study (103 women). The average time since disease onset was 6.25 years. Headache, anxiety and depression were responsible for considerable impact in the period prior to lockdown. The impact of headache was not aggravated during lockdown, but anxiety and depression did become significantly worse. Recently diagnosed participants reported higher rates of anxiety, depression, dizziness and diplopia; chronically diagnosed participants reported a higher frequency of cognitive symptoms. CONCLUSIONS: The clinical situation of the participants deteriorated during lockdown, although the differences were smaller than previously thought. This was probably because the baseline situation was more severe than expected. Further studies are needed to clarify the medium and long-term impact of the COVID-19 pandemic on patients with idiopathic intracranial hypertension.

TITLE: Impacto del confinamiento por COVID-19 en pacientes con hipertensión intracraneal idiopática en España.Introducción. El confinamiento nacional instaurado en España en marzo de 2020 como respuesta a la pandemia por la enfermedad por coronavirus 2019 (COVID-19) pudo tener un efecto en la situación clínica, el manejo de la enfermedad y el acceso a la atención médica en pacientes con hipertensión intracraneal idiopática. Pacientes y métodos. Se realizó un estudio observacional transversal por medio de una encuesta en línea. Se registraron la frecuencia y el impacto en la calidad de vida de la cefalea, los síntomas visuales, el mareo, los síntomas cognitivos, la diplopía, la ansiedad y la depresión. Se registró el tratamiento de la enfermedad y los retrasos experimentados en el acceso a la atención médica. Se agrupó a los participantes según el tiempo de evolución desde el diagnóstico. Resultados. Participaron 112 pacientes (103 mujeres). El tiempo medio de evolución de la enfermedad fue 6,25 años. La cefalea, la ansiedad y la depresión fueron responsables de un impacto considerable en el período previo al confinamiento. El impacto de la cefalea no empeoró durante el confinamiento, pero la ansiedad y la depresión empeoraron significativamente. Los participantes diagnosticados recientemente comunicaron mayores tasas de ansiedad, depresión, mareo y diplopía; los de evolución crónica, mayor frecuencia de síntomas cognitivos. Conclusiones. La situación clínica de los participantes se deterioró durante el confinamiento, aunque las diferencias fueron menores de lo que se pensaba. Esto se debió, probablemente, a que la situación basal era más grave de lo esperado. Son necesarios más estudios para aclarar el impacto a medio y largo plazo de la pandemia por COVID-19 en pacientes con hipertensión intracraneal idiopática.

Multiple Sclerosis Presenting as Intracranial Hypertension in the Setting of COVID-19 Infection (preprint)

Background Multiple sclerosis (MS) and Idiopathic Intracranial Hypertension (IIH) occur more commonly in women of childbearing age. There is cerebral spinal fluid (CSF) altered dynamics in both diseases causing them to have similar presentation at times. Coronavirus disease 19 (COVID-19) have also been reported to affect the Central Nervous System (CNS) of any age.Case Presentation: We report A-19-year-old male who initially presented with headaches, and fever and was diagnosed with COVID-19 infection. A few days later, he developed acute severe left eye pain, blurred vision, diplopia, and left ear tinnitus. The patient was found to have reduced visual acuity, left sixth nerve palsy, esotropia and asymmetric bilateral papilledema. Later, he developed left facial nerve palsy. MRI of the brain showed extensive demyelinating lesions. Lumbar puncture revealed significantly increased intracranial pressure (ICP) and positive oligoclonal bands.Conclusion This is a unique case of MS presenting with intracranial hypertension (IH) in the setting of COVID-19 infection that could have been the trigger for the MS clinical attack.

Secondary Headache Disorders: Approach, Workup, and Special Considerations for Select Populations.

Headache is one of the most common diagnoses in neurology. A thorough understanding of the clinical presentation of secondary headache, which can be life-threatening, is critical. This review provides an overview of the diagnostic approach to a patient with headache, including discussion of "red," "orange," and "green" flags. We emphasize particular scenarios to help tailor the clinical workup to individual circumstances such as in pregnant women, when particular attention must be paid to the effects of blood pressure and hypercoagulability, as well as in older adults, where there is a need for higher suspicion for an intracranial mass lesion or giant cell arteritis. Patients with risk factors for headache secondary to alterations in intracranial pressure, whether elevated (e.g., idiopathic intracranial hypertension) or decreased (e.g., cerebrospinal fluid leak), may require more specific diagnostic testing and treatment. Finally, headache in patients with COVID-19 or long COVID-19 is increasingly recognized and may have multiple etiologies.

The diagnostic dilemma of idiopathic intracranial hypertension in a child with acute lymphoblastic leukemia: COVID-19 or cytosine arabinoside?

BACKGROUND: Idiopathic intracranial hypertension is a rare neurological condition among children. Its manifestations vary from mild headaches to loss of vision. Although rare, COVID-19 infection and high dose cytosine arabinoside have been reported as risk factors for this neurological disorder. In patients with acute leukemia, idiopathic intracranial hypertension diagnosis is simple, but finding its etiology can be difficult. CASE PRESENTATION: We report a case of a 9-year-old boy with an ongoing treatment for T-acute lymphoblastic leukemia presenting with persistent headaches and diplopia. A diagnosis of idiopathic intracranial hypertension was retained based on clinical, imaging and laboratory findings. Due to its rarity, we describe its clinical and therapeutic features and highlight the challenging etiological dilemma between COVID-19 infection and high dose cytosine arabinoside administration. CONCLUSION: Persistent headache in a pediatric patient with leukemia can be due to many neurological disorders other than leukemic relapse. Given the improvement of the neurological symptoms after the SARS-CoV-2 PCR negativization and the successful re-introduction of high dose cytosine Arabinoside, the diagnosis of idiopathic intracranial hypertension associated with Covid-19 infection was withheld.

Increase in Pediatric Pseudotumor Cerebri Syndrome Emergency Department Visits, Inpatient Admissions, and Surgeries During the COVID-19 Pandemic.

BACKGROUND: Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It is unknown whether the increase in childhood obesity during the COVID-19 pandemic has affected the rates and characteristics of pediatric PTCS. METHODS: We conducted a retrospective study of children evaluated for PTCS (inpatient or emergency department) at our children's hospital before (March 19, 2015 to March 19, 2020) and during (March 20, 2020 to February 20, 2021) the pandemic. We compared the monthly number of inpatient and emergency department encounters for pediatric PTCS before and during the pandemic. In addition, anthropometric and ophthalmologic characteristics of children evaluated for pediatric PTCS before and during the pandemic were compared. RESULTS: A total of 36 encounters in the 5 years before the pandemic and 26 encounters in the 11 months during the pandemic were identified. The median monthly number of encounters for pediatric PTCS was significantly higher during the pandemic compared with the 5 years before the pandemic (2 vs 0, P = 0.0021). Compared with prepandemic patients, children evaluated during the pandemic were older (median age 16 vs 14 years, P = 0.02), with higher rates of obesity (85% vs 66%, P = 0.05) and lower likelihood of reporting Caucasian race (4% vs 31%, P = 0.02). Pandemic patients had worse presenting visual acuity (median logMAR 0.14 vs 0.05, P = 0.05) and were more likely to have fulminant presentation (23% vs 6%, P = 0.04) and require surgical intervention (23% vs 6%, P = 0.04). CONCLUSIONS: At our children's hospital, the rate of inpatient admissions and emergency department visits for pediatric PTCS increased during the pandemic. The severity of disease and frequency of surgical treatment also increased. Racial and ethnic minorities seem to be disproportionately affected. These changes may be related to increasing rates of childhood obesity during the pandemic.

Pseudotumor Cerebri Syndrome as a Neurologic Involvement of Multisystem Inflammatory Syndrome in Children: A Case Report.

BACKGROUND: Coronavirus disease 2019 may have neurological manifestations including meningitis, encephalitis, post-infectious brainstem encephalitis and Guillain-Barre syndrome. Neuroinflammation has been claimed as a possible cause. Here, we present a child with multisystem inflammatory syndrome in children (MIS-C) who developed pseudotumor cerebri syndrome (PTCS) during the disease course. CASE: A 11-year-old girl presented with 5 days of fever, headache and developed disturbance of consciousness, respiratory distress, conjunctivitis and diffuse rash on her trunk. Immunoglobulin M and G antibodies against severe acute respiratory syndrome coronavirus 2 were positive in her serum. She was diagnosed with MIS-C. On day 10, she developed headache and diplopia. Left abducens paralysis and bilateral grade 3 papilledema were observed. Brain magnetic resonance imaging revealed optic nerve head protrusion, globe flattening. She was diagnosed with secondary PTCS. Papilledema and abducens paralysis improved under acetazolamide and topiramate. Neurological examination became normal after 2 months. CONCLUSION: PTCS may emerge related to MIS-C.

Pediatric Intracranial Hypertension: A Spotlight on Imaging, the Idiopathic Intracranial Hypertension Treatment Trial, and COVID-19 Associated Cases.

Primary intracranial hypertension (PIH) is characterized by clinical signs of increased intracranial pressure, papilledema, elevated opening pressure, and absence of mass lesion, hydrocephalus, or meningeal enhancement on neuroimaging. Visual changes are a common presenting feature and if untreated there is risk of irreversible vision loss. There have been recent proposed changes to the criteria for PIH along with studies looking at the differences in imaging characteristics between adult and pediatric PIH. The presence of transverse sinus stenosis alone was highly sensitive and specific for pediatric PIH. The Idiopathic Intracranial Hypertension Treatment Trial was an adult, multicenter study that examined the use of acetazolamide and weight loss on the course of PIH. The study confirmed many previously held beliefs including the most common presenting symptom in PIH is headache. Most patients present with bilateral papilledema with 58.2% of patients having symmetric Frisen scale grading and within one grade in 92.8%. Although diplopia is a common reported symptom, very few have evidence of cranial nerve palsy. Male gender, high-grade papilledema, and decreased visual acuity at presentation are risk factors for treatment failure. Acetazolamide use is associated with mild metabolic acidosis. During acetazolamide treatment, monitoring for hypokalemia or aplastic anemia is not recommended. Monitoring transaminases in the titration phase of treatment should be considered due to a case of transaminitis and pancreatitis with elevated lipase. Newer case reports have also seen associations of secondary intracranial hypertension with concurrent COVID-19 infection and MIS-C.

Management of Idiopathic Intracranial Hypertension During the COVID-19 Pandemic.

BACKGROUND: In the current coronavirus disease 2019 (COVID-19) pandemic, health systems are struggling to prioritize care for affected patients; however, physicians globally are also attempting to maintain care for other less-threatening medical conditions that may lead to permanent disabilities if untreated. Idiopathic intracranial hypertension (IIH) is a relatively common condition affecting young females that could lead to permanent blindness if not properly treated. In this article, we provide some insight and recommendations regarding the management of IIH during the pandemic. METHODS: The diagnosis, follow-up, and treatment methods of IIH during the COVID-19 pandemic period are reviewed. COVID-19, as a mimic of IIH, is also discussed. RESULTS: Diagnosis and follow-up of papilledema due to IIH during the COVID-19 pandemic can be facilitated by nonmydriatic fundus photography and optical coherence tomography. COVID-19 may mimic IIH by presenting as cerebral venous sinus thrombosis, papillophlebitis, or meningoencephalitis, so a high index of suspicion is required in these cases. When surgical treatment is indicated, optic nerve sheath fenestration may be the primary procedure of choice during the pandemic period. CONCLUSION: IIH is a serious vision-threatening condition that could lead to permanent blindness and disability at a relatively young age if left untreated. It could be the first presentation of a COVID-19 infection. Certain precautions during the diagnosis and management of this condition could be taken that may allow appropriate care to be delivered to these patients while minimizing the risk of coronavirus infection.

COVID-19 and chronic fatigue syndrome: Is the worst yet to come?

There has been concern about possible long-term sequelae resembling myalgic encephalomyelitis/chronic fatigue syndrome in COVID-19 patients. Clarifying the mechanisms underlying such a "post-COVID-19 fatigue syndrome" is essential for the development of preventive and early treatment methods for this syndrome. In the present paper, by integrating insights pertaining to the glymphatic system and the nasal cerebrospinal fluid outflow pathway with findings in patients with chronic fatigue syndrome, idiopathic intracranial hypertension, and COVID-19, I provide a coherent conceptual framework for understanding the pathophysiology of post-COVID-19 fatigue syndrome. According to this hypothesis, this syndrome may result from damage to olfactory sensory neurons, causing reduced outflow of cerebrospinal fluid through the cribriform plate, and further leading to congestion of the glymphatic system with subsequent toxic build-up within the central nervous system. I further postulate that patients with post-COVID-19 fatigue syndrome may benefit from cerebrospinal fluid drainage by restoring glymphatic transport and waste removal from the brain. Obviously, further research is required to provide further evidence for the presence of this post-viral syndrome, and to provide additional insight regarding the relative contribution of the glymphatic-lymphatic system to it. Other mechanisms may also be involved. If confirmed, the glymphatic-lymphatic system could represent a target in combating post-COVID-19 fatigue syndrome. Moreover, further research in this area could also provide new insights into the understanding of chronic fatigue syndrome.

The diagnostic dilemma of NMOSD/anti-MOG Antibody and Pseudotumor cerebri co-existence in a patient with recent COVID-19 infection. (preprint)

SARS-CoV-2 infection may present with numerous neurological manifestations. We report the case of a young obese lady, recently discharged after treatment for COVID-19 infection, who later presented with headache and blurred vision, diagnosed to have pseudotumor cerebri and concurrent bilateral optic neuritis.

Change of disease distribution in pediatric neurology inpatients during the COVID-19 outbreak in southwest China (preprint)

This study aimed to investigate the change of disease distribution in pediatric neurology inpatients during the COVID-19 outbreak in southwest China. We retrospectively extracted the demographic data and diagnosis of discharged patients registered at pediatric neurology department of Chengdu Women’s and Children’s Central Hospital from January 1 to July 31, 2019 and January 1 to July 31, 2020. Total number of inpatients decreased during COVID-19 outbreak. Children diagnosed as febrile seizure caused by infection (1799/60.7% vs 980/59%, P = 0.141), dyskinesia (31/1.0% vs 28/1.7%, P = 0.075) and benign intracranial hypertension (41/1.4% vs 21/1.3%, P = 0.791) did not change. While children diagnosed as epilepsy (304/10.3% vs 348/21%, P < 0.001), migraine (25/0.8% vs 31/1.9%, P = 0.003), mental disease (24/0.8% vs 43/2.6%, P < 0.001) and peripheral neuropathy (38/1.3% vs 43/2.6%, P = 0.001) increased in 2020. Children diagnosed as intracranial infection (535/18% vs113/6.8%, P < 0.001) and myopathy (106/3.6% in vs 22/2.0%, P = 0.003) reduced in 2020. Conclusions: We found a significant increase in the proportion of mood-related diseases, while disease caused by infection decreased. We should pay attention to children’s mental state during the public health epidemic and the management of chronic disease.