Hemiplegic Migraine.

PURPOSE OF REVIEW: In this article, we review recent updates to the epidemiology, diagnostic testing, genetics, pathophysiology, and management of hemiplegic migraine. RECENT FINDINGS: While three genes have been historically associated with hemiplegic migraine, recent studies suggest two additional genes may also be implicated including PPRT2 and SLC1A3. Hemiplegic migraine is a severe subset of migraine with aura with symptoms including reversible hemiparesis in addition to other aura symptoms such as visual, sensory, or speech. The exact pathophysiology of hemiplegic migraine is not clear, but it is thought that this phenomenon is due to neuronal and glial depolarization causing cortical spreading depression. Due to the severity of presentation as well as the numerous mimickers, it is important to know a comprehensive differential and work-up. Given the low prevalence of the disease, most studies regarding treatment are limited to case studies. There is still an important need for further and larger studies regarding management of these cases.

Calcitonin gene relating peptide inhibitors in combination for migraine treatment: A mini-review.

The discovery of calcitonin gene-related peptide (CGRP) and its role in migraine pathophysiology has led to advances in the treatment of migraine. Since 2018, the Food and Drug Administration (FDA) has approved four monoclonal antibody (mab) therapies targeting either the CGRP ligand or receptor and 3 oral small molecule CGRP receptor antagonists. These targeted therapies have been shown to be safe and effective for either preventive or acute treatment of migraine in adults. Given their efficacy and tolerability profile, CGRP inhibitors have revolutionized the approach to migraine treatment. Theoretically, combining therapies within this therapeutic class could lead to more CGRP blockade and, subsequently, improved patient outcomes. There are providers currently combining CGRP therapies in clinical practice. However, limited data are available regarding the efficacy and safety of this practice. This mini-review provides a summary of available data and poses important considerations when combining CGRP therapies for migraine treatment.

Rituximab for Autoimmune Encephalitis with Epilepsy.

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.

Utilizing the Flexibility of Directional Deep Brain Stimulation Intraoperatively (if Needed) to Minimize Microelectrode Lead Repositioning.

This index case report describes the intraoperative use of an eight-contact directional deep brain stimulation (DBS) lead to avoid adjustment and repeat microelectrode passes after the initial pass elicited side-effects that suggested a slightly anteriorly placed lead. While targeting the subthalamic nucleus (STN), intraoperative microelectrode recording (MER) confirmed that lead positioning and macrostimulation resulted in response at 1 mA but sustained dysarthria at 2 mA. This suggested a slightly anteriorly located electrode. The patient was becoming anxious, so instead of lead adjustment, an eight-contact directional DBS lead was placed to take advantage of the directional contacts, noting that a repeat pass could always then be performed. Segmented contact 11C showed symptom response at 0.5 mA and side-effect at 4 mA, resulting in a 3.5 mA therapeutic window. Though no substitute for an accurately placed lead, this result suggests that the flexibility of directional stimulation could be considered in the intraoperative setting.

New Onset British Accent, Acute Behavioral Changes, and Seizures: A Unique Presentation of NMDAR Encephalitis.

The leading cause of autoimmune encephalitis is N-methyl-D-aspartate receptor (NMDAR) encephalitis. Symptoms can present as prominent behavioral abnormalities prompting inaccurate psychiatric diagnoses. Psychiatric features such as bizarre behavior, agitation, anxiety, delusions, and hallucinations are well noted in the current literature, but a manifestation of foreign accent syndrome has, to our knowledge, never been reported in cases of encephalitis. Once diagnosed, initiation of therapy can result in effective treatment. Here, we present a case of a 32-year-old female with new onset seizures and marked behavioral changes, such as speaking in a foreign accent, who was empirically treated for NMDAR encephalitis due to strong clinical suspicion, showed no improvement with first line therapy with IVIG and IV steroids, and finally had rapid resolution of symptoms with the early initiation of second line therapy of rituximab. In a young female presenting with nonspecific behavioral changes, NMDAR encephalitis should be on the differential and, although CSF antibodies are definitively diagnostic, there should be a low threshold to start empiric therapy and escalate to second line treatment.

Rare Case of Anti-LGI1 Limbic Encephalitis with New Onset Epilepsy: A Case Report.

Anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) has been classified as an autoimmune LE with a subacute course. Many patients with anti-LGI1 LE have normal or minimal cerebrospinal fluid (CSF) findings. Cerebrospinal fluid 14-3-3 protein or neuron specific enolase is usually seen in Creutzfeldt-Jakob disease (CJD) with high sensitivities, but can also be positive in other paraneoplastic and autoimmune encephalitides, which can make diagnosis challenging. The mainstay of treatment for anti-LGI1 LE generally focuses on steroids, intravenous immunoglobulin (IVIG), plasmapheresis, and/or rituximab. All the aforementioned modalities can be used in the treatment of anti-LGI1 LE and since this condition is highly responsive to treatment with steroids, prompt diagnosis can help stall the progression of this disease. Here, we present a case of anti-LGI1 LE that initially improved with empiric immunotherapy and showed definitive return to baseline with initiation of rituximab.

Unilateral Hyperkinetic Choreiform Movements due to Calcification of the Putamen and Caudate from an Underlying Developmental Venous Anomaly.

This index case report describes a patient who presented with unilateral hyperkinetic choreiform movements of the left neck, arm, and leg caused by right-sided putamen and caudate calcification as the product of an underlying developmental venous anomaly (DVA). No underlying metabolic disorder or other calcium-related disorder was present. Calcification of the putamen and caudate has been described in relation to an underlying DVA which results in localized venous hypertension and other changes, and tends to spare the anterior limb of the internal capsule. This resulting unilateral choreiform movement disorder has not been described in the literature and represents the need for greater recognition of this entity in the differential for lateralizing hyperkinetic disorders.