Chronic necrotizing granulomatous meningoencephalitis is an idiopathic inflammatory disease with possible autoimmune mediated delayed type hypersensitivity response. It commonly affects the central ...nervous system of dogs, and on rare occasions cats. However, this inflammatory disease has rarely been reported in humans. A 69-year-old woman presented with subacute intermittent sharp headaches. Brain MRI revealed multifocal enhancing lesions and edema as well as multifocal signal abnormality throughout the supratentorial and infratentorial parenchyma. Cerebrospinal fluid showed elevated opening pressure, pleocytosis, and elevated protein. MRA head, MRV head, cerebral angiogram, extensive serum and CSF work up for infectious and autoimmune etiologies were also unremarkable. Brain biopsy revealed necrotizing granulomatous inflammation with dystrophic calcification. High dose of methylprednisolone followed by a 6-week prednisone taper resolved her headache and the brain MRI abnormalities. This case demonstrates the response of necrotizing granulomatous meningoencephalitis in humans to steroids.
•Glioblastoma multiforme may present with atypical imaging features and can mimic many non-neoplastic processes.•Contrast enhancement on MRI can sometimes be absent in glioblastoma multiforme.•MR ...spectroscopy and brain biopsy are additional diagnostic tools that can help confirm the diagnosis of Glioblastoma when MRI is equivocal.
Glioblastoma is one of the most aggressive malignancies, and can present with atypical clinical and radiological features, resulting in delays in diagnosis. We report a case of glioblastoma multiforme, presenting with atypical radiological features mimicking herpes encephalitis. The patient was eventually diagnosed with glioblastoma after a brain biopsy with histopathological and molecular testing. We highlight and discuss diagnostic challenges and other supportive diagnostic tools when considering glioblastoma.
Acute arterial strokes in children are rare but can potentially cause lasting and often permanent neurological deficits. Mechanical thrombectomy has a well-established efficacy and safety profile in ...adult stroke management, but in the pediatric population, it is yet to be proven efficacious and safe. We present a case of a seven-year-old male who presented with multiple episodes of generalized tonic-clonic seizures after sustaining a neck injury by falling from a trampoline. National Institutes of Health (NIH) on presentation was 21. Neurological exam revealed dilated nonreactive pupils, dysconjugate gaze, severe dysarthria, bilateral ptosis, and movement of upper and lower extremities only to noxious stimuli. Magnetic resonance imaging (MRI) of brain without contrast revealed infarcted areas in the left pons, midbrain, and cerebellar regions. Computed tomographic angiogram (CTA) of head demonstrated left vertebral artery dissection with associated complete occlusion of the distal basilar artery. Successful recanalization was achieved with mechanical thrombectomy six hours after presentation. Mechanical thrombectomy treatment resulted in a significant neurological recovery with NIH of 1. This case supports the growing evidence of the efficacy and safety of mechanical thrombectomy in children.
Brain MRI in Status Epilepticus (SE) is often helpful in diagnosis, lateralization and localization of the seizure focus. MRI changes in SE include predominantly ipsilateral diffusion weighted ...imaging (DWI) changes in the hippocampus and pulvinar or similar changes involving basal ganglia, thalamus, cerebellum, brain stem and external capsule (Chatzikonstantinou et al., 2011 1). These changes are thought to be due to transient vasogenic and cytotoxic edema due to either transient damage or breakdown of blood brain barrier, proportional to the frequency and duration of the epileptic activity (Amato et al., 2001 2). Such changes may also be reflected on T2- weighted and T2-Fluid-Attenuated Inversion Recovery (FLAIR) sequences of MRI.
Herein, we present a case of a transient FLAIR cerebrospinal fluid (CSF) hyperintensity on the second MRI brain in a patient with focal status epilepticus. This imaging finding led to diagnostic confusion and was initially thought to represent subarachnoid hemorrhage. However, lumbar puncture, brain computed tomography (CT), and a follow-up brain MRI ruled out that possibility and other CSF pathologies. We concluded that the transient FLAIR changes in the second brain MRI were related to a rare imaging pitfall caused by Gadolinium enhancement of CSF on the FLAIR sequence, popularly referred to as hyperintense acute reperfusion marker (HARM).
•Postcontrast enhancement of the CSF on the FLAIR sequence can occur in seizures in a lateralizing fashion•Transient FLAIR signal in subarachnoid spaces in seizure may lead to a diagnostic confusion with subarachnoid hemorrhage•Renal insufficiency can precipitate this phenomenon in seizure patients•Early recognition of this MRI pitfall is paramount to avoid unnecessary testing
•Lance-Adam is an incredibly rare complication of successful cardiopulmonary resuscitation (CPR).•Electroencephalogram (EEG) can be vital in the diagnosis of LAS and allow for better prognostication ...and management in patients with post hypoxic myoclonus.•Polyspike-wave discharges were noted primarily or maximally at the vertex in our 5 cases of LAS.•Multiple anti-seizure medications are often required in the management of LAS.•Brain magnetic resonance imaging (MRI) abnormalities are not specific in LAS.
Lance-Adams Syndrome (LAS) is an incredibly rare complication of successful cardiopulmonary resuscitation (CPR). It is a form of posthypoxic myoclonus characterized by action or intention myoclonus developing days to months after an hypoxic insult to the brain. LAS, especially early in a patient's clinical course, can be challenging to diagnose. In this case series, all of the patients presented after achieving return of spontaneous circulation (ROSC) and subsequently developed myoclonus. Electroencephalogram (EEG) findings later showed changes consistent with LAS in all 5 patients. Our cases highlight the EEG characteristics that can be seen in LAS, which can aid in the diagnosis of LAS, as well as long term treatment outcomes of the LAS. We also reviewed the available literature to better understand the prevalence, mechanism, clinical presentation, diagnosis, and management of LAS.
Introduction:
Multiple risk factors of mortality have been identified in patients with COVID-19. Here, we sought to determine the effect of a history of neurological disorder and development of ...neurological manifestations on mortality in hospitalized patients with COVID-19.
Methods:
From March 20 to May 20, 2020, hospitalized patients with laboratory confirmed or highly suspected COVID-19 were identified at four hospitals in Ohio. Previous history of neurological disease was classified by severity (major or minor). Neurological manifestations during disease course were also grouped into major and minor manifestations. Encephalopathy, ischemic or hemorrhagic stroke, and seizures were defined as major manifestations, whereas minor neurological manifestations included headache, anosmia, dysgeusia, dizziness or vertigo, and myalgias. Multivariate logistic regression models were used to determine significant predictors of mortality in patients with COVID-19 infection.
Results:
574/626 hospitalized patients were eligible for inclusion. Mean age of the 574 patients included in the analysis was 62.8 (SD 17.6), with 298 (51.9%) women. Of the cohort, 240(41.8%) patients had a prior history of neurological disease (HND), of which 204 (35.5%) had a major history of neurological disease (HND). Mortality rates were higher in patients with a major HND (30.9 vs. 15.4%;
p
= 0.00002), although this was not a significant predictor of death. Major neurological manifestations were recorded in 203/574 (35.4%) patients during disease course. The mortality rate in patients who had major neurological manifestations was 37.4% compared to 11.9% (
p
= 2 × 10
−12
) in those who did not. In multivariate analysis, major neurological manifestation (OR 2.1, CI 1.3-3.4;
p
= 0.002) was a predictor of death.
Conclusions:
In this retrospective study, history of pre-existing neurological disease in hospitalized COVID-19 patients did not impact mortality; however, development of major neurological manifestations during disease course was found to be an independent predictor of death. Larger studies are needed to validate our findings.
Background
In December 2019, unexplained cases of pneumonia emerged in Wuhan, China, which were found to be secondary to the novel coronavirus SARS-CoV-2. On March 11, 2020, the WHO declared the ...Coronavirus Disease 2019 (COVID-2019) outbreak, a pandemic.
Objective
To clarify the neurological complications of SARS-CoV-2 infection including the potential mechanisms and therapeutic options.
Methods
We conducted a systematic literature search from December 01, 2019 to May 14, 2020 using multiple combinations of keywords from PubMed and Ovid Medline databases according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We included articles with cases of COVID-19 where neurological involvement was evident.
Results
We were able to identify 82 cases of COVID-19 with neurological complications. The mean age was 62.3 years. 37.8% of the patients were women (
n
= 31). 48.8% of the patients (
n
= 40) had cerebrovascular insults, 28% (
n
= 23) had neuromuscular disorders, and 23% of the patients (
n
= 19) had encephalitis or encephalopathy.
Conclusions
Neurological manifestations of COVID-19 are not rare, especially large vessel stroke, Guillain–Barre syndrome, and meningoencephalitis. Moving forward, further studies are needed to clarify the prevalence of the neurological complications of SARS-CoV-2 infection, investigate their biological backgrounds, and test treatment options. Physicians should be cautious not to overlook other neurological diagnoses that can mimic COVID-19 during the pandemic.
Intracranial hypotension is a result of low Cerebrospinal fluid (CSF) pressure caused by either spontaneous or postoperative leakage. The classic presentation of spontaneous intracranial hypotension ...is acute orthostatic headache, but the diagnosis can sometimes be challenging as some patients may present with atypical initial presentations including cervical pain as well as cervical radiculopathy secondary to cervical spine venous engorgement. We described a 42-year-old female patient who presented initially with neuropathic pain symptoms as well as weakness involving both lower extremities for which she underwent diagnostic lumbar puncture with concern regarding demyelinating neuropathy. However, subsequently she developed postural headache as well as severe cervical pain which was attributed to cervical epidural venous engorgement in setting of intracranial hypotension based on cervical spine magnetic resonance imaging (MRI) findings. She was managed conservatively, and repeated cervical spine MRI 3 days later showed prominent improvement in the imaging findings. Spinal epidural venous engorgement can occur secondary to intracranial hypotension (mainly post lumbar puncture), and can present clinically with neck pain or even symptoms of radiculopathy. Since the findings can mimic more serious conditions, it is extremely important to consider this condition in the differential diagnosis of an enhancing epidural collection in the cervical spine, particularly when intracranial hypotension is suspected.
In patients with cystic fibrosis (CF) and asthma, elevated levels of interleukin-8 (IL-8) are found in the airways. IL-8 is a CXC chemokine that is a chemoattractant for neutrophils through CXCR1 and ...CXCR2 G protein-coupled receptors. We hypothesized that IL-8 acts directly on airway smooth muscle cells (ASMC) in a way that may contribute to the enhanced airway responsiveness and airway remodeling observed in CF and asthma. The aim of this study was to determine whether human ASMC (HASMC) express functional IL-8 receptors (CXCR1 and CXCR2) linked to cell contraction and migration. Experiments were conducted on cells harvested from human lung specimens. Real-time PCR and fluorescence-activated cell sorting analysis showed that HASMC expressed mRNA and protein for both CXCR1 and CXCR2. Intracellular Ca(2+) concentration (Ca(2+)(i)) increased from 115 to 170 nM in response to IL-8 (100 nM) and decreased after inhibition of phospholipase C (PLC) with U-73122. On blocking the receptors with specific neutralizing antibodies, changes in Ca(2+)(i) were abrogated. IL-8 also contracted the HASMC, decreasing the length of cells by 15%, and induced a 2.5-fold increase in migration. These results indicate that HASMC constitutively express functional CXCR1 and CXCR2 that mediate IL-8-triggered Ca(2+) release, contraction, and migration. These data suggest a potential role for IL-8 in causing abnormal airway structure and function in asthma and CF.
Isaac syndrome (IS) is a peripheral nerve hyperexcitability state associated with voltage-gated potassium channel (VGKC) complex antibodies. Major manifestations are muscle twitching, stiffness, ...hypertrophy, and dysautonomic features such as hyperhidrosis Ahmed and Simmons. Muscle Nerve. 2015;52(1):5–12. Neuropathic pain is a rare manifestation. We describe a case of IS characterized by muscle twitching and intractable neuropathic pain. Diagnostic workup included elevated VGKC complex antibodies and EMG/NC that showed neuromyotonic discharges. Neuropathic pain was initially difficult to relieve even after using multiple medications, including opiates, benzodiazepines, anticonvulsants, and intravenous immunoglobulin (IVIg). Moderate pain control was eventually achieved with long-term use of carbamazepine and subcutaneous immunoglobulin (SCIg). Common manifestations of IS are muscle twitching, stiffness hypertrophy, and dysautonomia Ahmed and Simmons. Muscle Nerve. 2015;52(1):5–12. Sensory manifestations such as neuropathic pain are rare, but, as illustrated by our patient, can be the most distressing symptom. In our patient, not only was neuropathic pain disabling but it also showed the least response to IVIg. The use of 200 mg of long-acting carbamazepine twice daily with weekly SCIg demonstrated the best response. This case highlights an uncommon but potentially resistant symptom of IS.
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