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.

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.

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).

Malignant Hyperthermia.

Malignant Hyperthermia (MH) is a life-threatening pharmacogenetic disorder which results from exposure to volatile anesthetic agents and depolarizing muscle relaxants. It manifests as a hypermetabolic response resulting in tachycardia, tachypnea, hyperthermia, hypercapnia, acidosis, muscle rigidity and rhabdomyolysis. An increase in the end-tidal carbon dioxide is one of the earliest diagnostic signs. Dantrolene sodium is effective in the management of MH, and should be available whenever general anesthesia is administered. This review also aims to highlight the genetics and pathology of MH, along with its association with various inherited myopathy syndromes like central core disease, multi-mini core disease, Native-American myopathy, and King-Denborough syndrome.

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.

Leucoencephalopathy with brain stem and spinal cord involvement and lactate elevation: a novel mutation in the DARS2 gene.

Leucoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a very rare autosomal recessive, slowly progressive neurological disorder characterised by distinctive clinical findings including cerebellar, pyramidal and dorsal column dysfunction. This is caused by a mutation in the DARS2 gene, which encodes mitochondrial aspartyl-tRNA synthetase. MRI shows distinctive abnormalities in the cerebral white matter and specific brain stem and spinal cord tracts. Here, we present a case of LBSL, with a novel c.1192-2A>G mutation.

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.

Glia Maturation Factor and Mast Cell-Dependent Expression of Inflammatory Mediators and Proteinase Activated Receptor-2 in Neuroinflammation.

Parkinson's disease (PD) is characterized by the presence of inflammation-mediated dopaminergic neurodegeneration in the substantia nigra. Inflammatory mediators from activated microglia, astrocytes, neurons, T-cells and mast cells mediate neuroinflammation and neurodegeneration. Administration of neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induces PD like motor deficits in rodents. 1-methyl-4-phenylpyridinium (MPP+), a toxic metabolite of MPTP activates glial cells, neurons and mast cells to release neuroinflammatory mediators. Glia maturation factor (GMF), mast cells and proteinase activated receptor-2 (PAR-2) are implicated in neuroinflammation. Alpha-synuclein which induces neurodegeneration increases PAR-2 expression in the brain. However, the exact mechanisms are not yet understood. In this study, we quantified inflammatory mediators in the brains of MPTP-administered wild type (Wt), GMF-knockout (GMF-KO), and mast cell knockout (MC-KO) mice. Additionally, we analyzed the effect of MPP+, GMF, and mast cell proteases on PAR-2 expression in astrocytes and neurons in vitro. Results show that the levels of interleukin-1beta (IL-1ß), tumor necrosis factor-alpha (TNF-α), and the chemokine (C-C motif) ligand 2 (CCL2) were lesser in the brains of GMF-KO mice and MC-KO mice when compared to Wt mice brain after MPTP administration. Incubation of astrocytes and neurons with MPP+, GMF, and mouse mast cell protease-6 (MMCP-6) and MMCP-7 increased the expression of PAR-2. Our studies show that the absence of mast cells and GMF reduce the expression of neuroinflammatory mediators in the brain. We conclude that GMF along with mast cell interactions with glial cells and neurons during neuroinflammation can be explored as a new therapeutic target for PD and other neuroinflammatory disorders.

Infusion-rate-dependent Acute Neuropathic Pain with Duopa in a Patient with Parkinson's Disease and Pre-existing Neuropathy.

The association of peripheral neuropathy (PN) with the cumulative use of levodopa, presumably by vitamin B12 deficiency, has been well-documented in the literature. However, less is known about the L-dopa infusion-rate-dependent precipitation of pre-existing peripheral neuropathy. We report an unusual case of Parkinson's disease in a patient who presented with acute exacerbations of pre-existing peripheral neuropathy on exceeding certain rates of continuous Carbidopa-Levodopa Infusions (CLI). We present a case of a 68-year-old gentleman with a 20-year history of idiopathic Parkinson's disease with bilateral subthalamic nucleus deep brain stimulation who was started on Duopa therapy for worsening dyskinesias. Workup before initiating Duopa was significant for idiopathic sensorimotor axonal polyneuropathy, although the symptoms were well controlled with medications. Subsequently, the patient developed severe neuropathic pain within 24 hours of initiating Duopa characterized by burning and stinging in his feet. Symptoms resolved within four hours of reducing the continuous infusion rates without modifying the bolus doses. The patient was doing well with extra bolus doses to manage off periods with no further recurrence of symptoms. With the support of this case report, we would like to conclude that the acute worsening of neuropathic pain with infusion rate or duration of treatment might limit the clinical benefits of Duopa and adds to the expanding spectrum of neurotoxic side effects associated with this therapy. Further prospective studies are needed to monitor the acute worsening of neuropathic pain in patients with pre-existing PN, after initiating CLI and its association with doses of levodopa/carbidopa.

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.

An Unusual Case of Antiphospholipid Syndrome Presenting as Chorea.

Movement disorders are rarely reported in association with antiphospholipid syndrome (APS). Although it is a rare manifestation in APS, chorea is the most common movement disorder. We report a case of APS in a patient who presented with hyperkinesia, tics, choreiform movements, and other dyskinetic movements involving the mouth and tongue along with behavioral changes. These abnormal movements improved with aripiprazole.