Neuropsychiatric Manifestations of Autoimmune Encephalitis in a Tertiary Hospital: A Case Series and Current Perspectives.

Importance: Autoimmune encephalitis (AE) refers to a group of neuropsychiatric conditions associated with specific circulating autoantibodies directed against synaptic receptors, neuronal cell surface proteins, and intracellular targets. Increased recognition of these disorders is of value, as affected patients prominently display cognitive impairment, behavioral disturbances, and seizures requiring multidisciplinary teams, with early recognition often impacting prognosis.Observations: This case series is based on a retrospective record review of adult patients diagnosed with AE between January 1, 2010- January 1, 2020. Cases 1 and 2, demonstrating anti- N-methyl-d-aspartate receptor (NMDAR) encephalitis with initial manifestations of neurologic and psychiatric symptoms, correlate with the literature describing a higher prevalence of this condition in young women. Case 3, despite being seronegative, exhibited classic features of anti-NMDAR encephalitis. Case 4 demonstrates a classic presentation of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis: a middle-aged male with psychosis, altered mentation, and epilepsy. Case 5 had a more indolent neuropsychiatric presentation with mild elevation of N-type voltage-gated potassium channel (VGKC) antibody. Case 6, with glutamic acid decarboxylase 65 (GAD65) antibody, was an elderly female with speech dysfunction and altered mentation, and case 7 was an elderly male with GAD65 antibody who had stiff-person syndrome, ataxia, cognitive decline, and thymoma.Conclusions: This retrospective case series describes the clinical details of 7 individuals with AE and overlapping neuropsychiatric symptoms. This series is limited in scope, with a small number of cases and observational findings, which prevents specific conclusions from being drawn. Despite this limitation, the present article explores the nuances of variable presentations of this disease to inform better interdisciplinary management and emphasize the gap areas that need rigorous research.

Infectious Causes of Stroke.

Stroke is one of the primary causes of mortality and morbidity worldwide. It can be ischemic or hemorrhagic, and the former can be due to an in-situ thrombus or a distant embolus. Despite being a rare cause, stroke can also be caused in the setting of infection. Bacterial agents are the most common cause of stroke, among other infectious agents. Until the antibiotic era, rheumatic heart disease was a predisposing risk factor of infective endocarditis. VZV is the most common cause of strokes in pediatric and adult populations. Cryptococcus and Candida spp are the most common yeasts involved in CNS infections, especially in immunocompromised patients. In COVID-19 patients, ischemic strokes are more common than hemorrhagic strokes. In this review, we will discuss the most common infectious agents, with particular emphasis on COVID-19.

Hereditary spastic paraplegia.

Hereditary spastic paraplegias (HSPs) are a group of neurodegenerative disorders which involve the corticospinal tracts and present with distinct spasticity and weakness of the lower extremities. The estimated prevalence of HSP is around 1.8/100,000 cases for both autosomal dominant and autosomal recessive types. Classification of HSP is based on inheritance pattern, clinical phenotype, and molecular pathophysiological mechanisms. The most common neuropathological sign is the axonal degeneration involving the lateral corticospinal tracts in both the cervical and thoracic spinal cord. The target of this review article is to provide a comprehensive overview of the HSP classification, neuropathology, and differential diagnosis.

Hematoma Risk After Needle Electromyography in Patients Using Newer Oral Anticoagulants.

PURPOSE: To assess the safety of needle electromyography in patients on non-vitamin K oral anticoagulants (NOACs) compared with warfarin. METHODS: A retrospective chart review was done in patients who underwent needle electromyography studies while they were using warfarin and NOACs. After the needle electromyography, all the patients were monitored for 2 hours and ultrasound of high-risk muscle groups was done. The complications were classified based on the International Society on Thrombosis and Hemostasis definitions. RESULTS: Fifty-eight patients were included: 29 were using NOACs and the other 29 were on warfarin. The mean age was 59.33 ± 16 years. Hemorrhagic complications from needle electromyography were noted in 9 patients: 7 (77.7%) NOACs and 2 (22.3%) warfarin. Among them, 6 patients (66.6%) met the diagnostic criteria for Clinically Relevant Non-Major Bleeding criteria proposed by International Society on Thrombosis and Hemostasis and 3 patients (33.4%) had an asymptomatic hematoma on ultrasound evaluation. A total of 267 muscles were tested and only 9 (3.3%) muscles had hemorrhagic complications. One patient (rivaroxaban) had acute bleeding requiring pressure bandage, five patients (two apixaban, two rivaroxaban, and one warfarin) had clinical hematoma that required ice packs, and three patients (two rivaroxaban and one warfarin) had a hematoma on ultrasound of deep muscles. CONCLUSIONS: Patients on NOACs had minimal risk of clinically relevant hemorrhagic complications, and the risk is not significantly different from those on warfarin.

Primary Central Nervous System Lymphoma Presenting as Parkinsonism with Atypical MRI Findings and Elevated 14-3-3 Protein.

Primary central nervous system lymphoma (PCNSL) is a rare neoplasm with an incidence of 2 to 3% of all CNS malignancies. The diagnosis can be challenging, especially with atypical presentations. Movement disorders can be one of the rare presentations of PCNSL. Here, we present an unusual case of gradually progressing Parkinsonism with an elevation of cerebrospinal fluid (CSF) 14-3-3 protein and atypical imaging findings found to have PCNSL. A 76-year-old female patient presented with gait and intermittent speech difficulty. Initial workup revealed a contrast-enhancing lesion in the bilateral putamen and head of caudate without any mass effect. Her symptoms were rapidly progressed over 6 months and presented with mild dysarthria, bradykinesia, mild rigidity, and reduced left arm swinging. These features were consistent with Parkinsonism. The repeat imaging showed the progression of hyperintensities in the bilateral putamen. The patient underwent a stereotypic biopsy of the right caudate nucleus, which revealed PCNSL. She was treated with high-dose methotrexate and is currently in remission. Diagnosis of movement disorders remains clinical and rapid progression of symptoms, and atypical presentation must warrant further imaging and workup.

Painless Legs and Moving Toes Presenting as a Tardive Phenomenon: Case Report and Review of the Literature.

Painless legs and moving toes syndrome is a rare movement disorder. Various conditions like spinal cord trauma, lumbar radiculopathy, peripheral neuropathy, neuroleptic treatment, or traumatic lesions of the soft tissues of the foot can present with this disorder. In our case report, we aim to report a case of a young woman who developed painless legs and moving toes in the setting of chronic neuroleptic usage, which improved after the discontinuation of antipsychotics and treatment with clonazepam. We describe in detail her presentation and clinical examination, along with the review of the literature.

Hyperglycaemic chorea-ballism or unmasking of Huntington's chorea in a patient with diabetes.

Chorea-ballism is a neurological syndrome characterised by violent involuntary movements of one or both extremities. In the last decades, several patients with these involuntary movements were reported in association with hyperglycaemia. Here, we present a unique case of possible Huntington's disease, which could have been unmasked by the hyperglycaemic insult to the basal ganglia in a 64-year-old man who presented with chorea-ballism.

Is Magnetic Resonance Imaging Diffusion Restriction of the Optic Disc Head a New Marker for Idiopathic Intracranial Hypertension?

Background Idiopathic intracranial hypertension (IIH) is a headache syndrome due to raised intracranial pressure of unknown etiology. Before making the diagnosis of IIH, secondary causes of raised intracranial pressure must be ruled out. The radiological features associated with this condition have variable sensitivity and specificity. In this case series, we aim to describe a potential new radiological marker of IIH, that is, diffusion restriction, in the optic disc head and propose that this can be a specific finding in the appropriate clinical picture. Importance IIH causes vision loss and disabling daily headaches. The diagnosis of this condition is based on history and physical examination findings. Magnetic resonance imaging (MRI) is used to exclude other causes, but specific radiological markers for the diagnosis of IIH are lacking. Observations Five patients presented with the main complaint of headache, which was associated with blurry vision. All of our patients had a formal neuro-ophthalmological evaluation that confirmed the presence of optic disc edema in both eyes. They also underwent an MRI of the brain that showed diffusion restriction in the optic nerve head in either eye or both eyes. Patients underwent lumbar puncture in the lateral decubitus position, which revealed cerebrospinal fluid opening pressures > 25 cm H 2 O. They all responded well to standard treatments, with the resolution of symptoms in their follow-up appointments. Conclusion and Relevance The MRI diffusion restriction in the optic nerve head may be a reliable noninvasive marker for the diagnosis of IIH in the appropriate clinical picture.

White Matter Hyperintensities in Patients with Sporadic Hemiplegic Migraine.

BACKGROUND AND PURPOSE: To identify the differences in overall occurrence, location, and disease burden of white matter hyperintensities (WMH) in patients with sporadic hemiplegic migraine (SHM) and patients with migraine headaches. METHODS: We included patients who met diagnostic criteria proposed by the third International Classification of Headache Disorders (ICHD-3) for SHM and migraine headache. WMHs were identified using T2 fluid-attenuated inversion recovery axial sequence and classified based upon the location. The disease burden was assessed using Scheltens visual rating scale. RESULTS: Fifty patients met the diagnostic criteria for SHM and 100 patients for migraine headache. Patients in the study group were similar to the control group in terms of age (47.7 ± 12.2 years vs. 48.17 ± 9.7 years; P = .814) and gender (M: F; 14:36 vs. M: F 25:75; P = .693). WMH were found in 28 (56%) patients with SHM and 44 (44%) in patients with migraine headache. The proportion of patients with WMH was not different between the two groups (P = .166). On univariate analysis, the proportion of patients with WMH in parietal, occipital, and infratentorial regions was higher in patients with SHM. White matter burden determined by visual rating scale and proportion of patients with lesions ≥5 mm in diameter was also significantly higher in patients with SHM. On multivariate analysis, the WMH occurrence in the parietal lobe (P = .043) was found to be significantly higher in SHM. CONCLUSIONS: The WMH occurrence in patients with SHM is significantly more in the parietal lobe when compared to those with migraine headaches. WMH burden was also higher in patients with SHM, and larger white matter lesions occurred more frequently in these patients with SHM (compared to ordinary migraineurs).

Rapidly progressive global cerebral atrophy in the setting of anti-LGI1 encephalitis.

A 47-year-old man presented with complaints of breakthrough seizures, psychiatric and behavioural changes and catatonic features. MRI of the brain showed mild cerebral and right hippocampal atrophy, while the electroencephalogram showed intermittent right temporal slowing. With a presumed diagnosis of autoimmune encephalitis, he was treated with intravenous immunoglobulin (IVIG) and methylprednisolone, which significantly improved the symptoms. Serological testing later was positive for antileucine-rich glioma inactivated 1 antibody. Two months after the initial presentation, patient had a relapse of the symptoms without any further episodes of seizures. Repeat MRI of the brain showed a significant rapidly progressive diffuse cortical atrophy and hippocampal atrophy, more prominent on the right side along with hydrocephalus ex vacuo when compared with the initial MRI. He is currently on monthly IVIG therapy. At 4 months follow-up from the second imagining study, the patient had persistent MRI findings.

Sleep Medicine: Insomnia and Sleep.

Insomnia disorder is an economic burden and public health concern affecting up to one-third of the population of the United States. It is mostly seen in older age groups, and often considered a normal aging phenomenon. The diagnosis and treatment of insomnia rely mainly on a thorough sleep history to address the precipitating factors as well as maladaptive behaviors resulting in poor sleep. It is important for clinicians to recognize and manage the symptoms of insomnia to prevent the morbidity associated with it. This review aims to highlight the pathophysiology, associated comorbidities, clinical evaluation and effective management strategies for insomnia disorder.

Mononeuropathy due to Entrapment of Dorsal Cutaneous Peroneal Nerve: Clinical, Electrophysiological, and Imaging Findings.

Peroneal neuropathy is the most common mononeuropathy encountered in the lower extremities. Isolated injuries to the dorsal cutaneous peroneal nerve (DCPN) are uncommon, and most of the reported cases are due to trauma or iatrogenic causes. We report a case of a middle-aged woman who presented with a nine-month history of tingling sensation over the dorsum of her left foot with normal electromyography (EMG) findings and was subsequently diagnosed with entrapment of the DCPN at the ankle by ultrasonographic examination.

Horner Syndrome Due to Spontaneous Internal Carotid Artery Dissection.

Horner syndrome is a constellation of neurological findings consisting of ipsilateral ptosis, miosis, and anhidrosis. Partial Horner syndrome, comprising ipsilateral ptosis and miosis in the absence of anhidrosis, is a well-documented but uncommon manifestation of internal carotid artery dissection. We report the case of a 42-year-old male patient who presented with ipsilateral ptosis and miosis and was subsequently diagnosed with internal carotid artery dissection. In this case report, we discuss the anatomy of the oculosympathetic pathway and the pharmacological diagnosis for a better understanding of the localization of the lesions causing Horner syndrome.

Rett Syndrome in Males: A Case Report and Review of Literature.

Rett syndrome (RTT) is a neurodevelopmental disorder in which a period of normal development is followed by regression of previously acquired skills. RTT was originally thought to be present exclusively in females. However, advances in genetic testing and phenotypic identification revealed that it is not a female-only disorder as cases of males with similar phenotype were reported. RTT was considered lethal in males as it has an X-linked dominant inheritance. The purpose of this review is to report a case of RTT in young male and elaborate genetics and phenomenology of this disorder in males.

A Rare case of recurrent Guillain-Barré syndrome without albuminocytological dissociation.

Guillain-Barré syndrome (GBS) is an immune-mediated polyneuropathy, often preceded by an illness. It is a self-limiting illness in most of the cases, but recurrence is rare and can be seen in about 1%-6% of patients. GBS is characterised by progressive, symmetrical, proximal and distal weakness. Areflexia and sensory disturbances are also common. Patients with GBS usually have albuminocytological dissociation on cerebrospinal fluid (CSF) analysis. This is a case of a 69-year-old woman with recurrent GBS and normal CSF findings.

Hypersomnia.

Adequate alertness is necessary for proper daytime functioning. Impairment of alertness or increase in sleepiness results in suboptimal performance and adversely affects the quality of life. While some causes of somnolence are intrinsic to the brain circuitry and neurochemical architecture, others are due to maladaptive behaviors and disorders affecting the normal sleep homeostasis. Identification of the problem and understanding the underlying etiology is the key to timely treatment and better outcomes.

Sleep Medicine: Parasomnias.

Parasomnias are abnormal and undesirable behaviors during sleep and are thought to be due to the sleep state instability. Some of them are benign, while some of them point to a possible underlying neurodegenerative process. This article briefly discusses the clinical characteristics, demographics, and pathophysiology of major parasomnias and associated disorders. The classification outlined in this article conforms to the current version of International Classification of Sleep disorders.

Sleep Medicine: Restless Legs Syndrome.

Restless Legs Syndrome is a highly prevalent sensorimotor disorder characterized by urge to move the legs due to discomfort that primarily happens in the evening or at nights. Although the exact pathophysiology remains unclear, brain iron deficiency and altered dopaminergic function appears to play an important role in the pathogenesis of this condition. This disorder affects women more frequently and is associated with significant morbidity.

Delayed Hypoxic Reversible Leukoencephalopathy Syndrome.

We report a case of postanoxic leukoencephalopathy in a patient who started to have cognitive and behavioral changes weeks after the anoxic insult along with white matter lesions on neuroimaging and demyelination on brain biopsy. His disease course followed a steady decline initially both clinically and radiologically and assumed a steady plateau. Months after his decline, the patient was seen to be completely functional with substantially improved mental status examination and resolution of white matter changes on imaging. The course of this disease entity usually assumes a plateau after clinical worsening with little improvement subsequently. However, our patient showed a dramatic recovery to his baseline after a few months. In this article, we review mechanisms, presentation and the sequelae of hypoxic injury to the brain.

Wernicke's Encephalopathy as a Rare Complication of Hyperemesis Gravidarum: A Case Report and Review of Literature.

Wernicke's encephalopathy (WE) is a rare neurological syndrome that presents in the setting of thiamine deficiency. Though alcoholism is the most common cause of this condition, a few other etiologies include malnutrition from other causes, hemodialysis, and hyperemesis gravidarum. In this case report, we aim to report a case of a young woman who developed WE in the setting of hyperemesis gravidarum (HG) that improved with thiamine replacement. This manuscript details her presentation and clinical examination and includes a spontaneous upbeat nystagmus and goes over the condition along with a review of the literature.