Motor Aura Research Articles

DIAGNOSIS OF FAMILIAL HEMIPLEGIC MIGRAINE IN AN INTUBATED PREGNANT PATIENT WITH GLASGOW COMA SCORE OF 3

Familial hemiplegic migraine (FHM) is a rare disease that manifests as hemiparesis and is characterized by attacks of marked motor aura, often accompanied by seizures, nystagmus, ataxia, and altered consciousness. Patients with a diagnosis of FHM may also have coma and subsequent respiratory failure requiring intubation. In this case report; FHM diagnosis process of the patient who was admitted to the reanimation intensive care unit after the cesarean section is described. In this process, the importance of detailed anamnesis obtained from the patient's relatives during the reanimation intensive care unit period in the diagnosis of FHM was emphasized, as the diagnostic imaging and laboratory tests performed on the patient were normal.

Diagnostic challenges in sporadic hemiplegic migraine: a case report

Case Presentation
 Female, 62 years old, history previous of hypertension and multiple cavernomatosis, admitted to our service emergency room with hypothesis of stroke. The patient presented headache associated with nausea and phosphenes in the right eye, with evolution for right hemiplegia. However, she showed complete and spontaneous reversal of symptoms, in a few minutes. The patient presented similar symptoms with annual recurrence since 2019, always following migraine attack. She denied familiar history of migraine. Brain MRI showed multiple cavernomatosis, with no signs of old or recent ischemia. Brain and cervical CT angiography and laboratorial tests were normal. She was then diagnosed with sporadic hemiplegic migraine, with no family report of the disease. She received treatment with Amitriptyline 50 mg per day, without further recurrences.
 Discussion
 Hemiplegic migraine (HM) is a rare form of migraine with motor aura, which includes fully reversible motor weakness. The aura of HM is most probably caused by cortical spreading depression, a self-propagating wave of neuronal and glial depolarization that spreads across the cerebral cortex. Patients who are the first member of their family to have hemiplegic migraine are classified as having sporadic hemiplegic migraine. Some cases of sporadic hemiplegic migraine are caused by one of the genetic variants that cause familial hemiplegic migraine. The treatment of HM is empirical and mainly relies on principles of management of the common types of migraine, except for triptans use, medication historically contraindicated because of vasoconstrictor properties.
 Final comments
 Hemiplegic migraine is an important differential diagnosis with stroke in patients with migraine. Correct recognition of this condition can be a crucial factor in the treatment and prognosis of the patient in the emergency room.
 
 Keywords: hemiplegic migraine, motor aura, stroke mimics.

A Different Kind of Hemiplegic Migraine Gene

The key clinical feature of hemiplegic migraine (HM) is motor aura symptomatology. The diagnosis is confirmed by the finding of autosomal dominantly

Acute prolonged motor aura resembling ischemic stroke after COVID \u2212\u200919 vaccination (CoronaVac): the first case report

BackgroundWe report the first case of a patient who suffered transient focal neurological deficit mimicking stroke following CoronaVac vaccination. However, instead of an ischemic stroke, motor aura was suspected.Case presentationsA 24 year-old Thai female presented with left hemiparesis fifteen minutes after receiving CoronaVac. She also had numbness of her left arm and legs, flashing lights, and headaches. On physical examination, her BMI was 32.8. Her vital signs were normal. She had moderate left hemiparesis (MRC grade III), numbness on her left face, arms, and legs. Her weakness continued for 5 days. A brain CT scan was done showing no evidence of acute infarction. Acute treatment with aspirin was given. MRI in conjunction with MRA was performed in which no restricted diffusion was seen. A SPECT was performed to evaluate the function of the brain showing significant hypoperfusion of the right hemisphere. The patient gradually improved and was discharged.DiscussionsIn this study, we present the first case of stroke mimic after CoronaVac vaccination. After negative imaging studies had been performed repeatedly, we reach a conclusion that stroke is unlikely to be the cause. Presumably, this phenomenon could possibly have abnormal functional imaging study. Therefore, we believed that it might be due to cortical spreading depression, like migraine aura, which we had conducted a literature review.

From Genotype to Phenotype: Expanding the Clinical Spectrum of CACNA1A Variants in the Era of Next Generation Sequencing.

Ion channel dysfunction is a key pathological substrate of episodic neurological disorders. A classical gene associated to paroxysmal movement disorders is CACNA1A, which codes for the pore-forming subunit of the neuronal calcium channel P/Q. Non-polyglutamine CACNA1A variants underlie familial hemiplegic ataxia type 1 (FHM1) and episodic ataxia type 2 (EA2). Classical paroxysmal manifestations of FHM1 are migraine attacks preceded by motor aura consisting of hemiparesis, aphasia, and disturbances of consciousness until coma. Patients with EA2 suffer of recurrent episodes of vertigo, unbalance, diplopia, and vomiting. Beyond these typical presentations, several reports highlighted manifold clinical features associated with P/Q channelopathies, from chronic progressive cerebellar ataxia to epilepsy and psychiatric disturbances. These manifestations may often outlast the burden of classical episodic symptoms leading to pitfalls in the diagnostic work-up. Lately, the spreading of next generation sequencing techniques linked de novo CACNA1A variants to an even broader phenotypic spectrum including early developmental delay, autism spectrum disorders, epileptic encephalopathy, and early onset paroxysmal dystonia. The age-dependency represents a striking new aspect of these phenotypes und highlights a pivotal role for P/Q channels in the development of the central nervous system in a defined time window. While several reviews addressed the clinical presentation and treatment of FHM1 and EA2, an overview of the newly described age-dependent manifestations is lacking. In this Mini-Review we present a clinical update, delineate genotype-phenotype correlations as well as summarize evidence on the pathophysiological mechanisms underlying the expanded phenotype associated with CACNA1A variants.

V.A.Muratov. To pathological physiology and differential diagnosis of cortical epilepsy. Medical Review. 1896, n ° 1

The author cites the history of the illness of one patient with manifestations of cortical epilepsy: convulsions began on the face, then passed on to the arm and leg, for the most part the seizure was limited to one left side. Left n. facialis was paralyzed; in the left extremities - paresis with reduced rigidity and increased tendon reflexes, while in the arms paresis and rigidity were almost the same, and rigidity prevailed in the legs. After the seizure, the paralysis intensified sharply. Some seizures were preceded by a motor aura: clonic convulsions in the paralyzed limbs. Left side sensitivity was decreased; muscle feeling weakened, stronger in the arms, less in the legs. The patient died of tuberculous pneumonia. When opened, found in the right hemisphere softening, which occupied the lower third of the posterior central gyrus. Gyrus marginalis in the anterior part was destroyed. The softening into the depths did not extend equally. In the rear part, it went quite deep; the radiant end in the supramargi nalis area is softened, the system of long connecting paths, apparently, is not affected; small focus in the superior parietal gyrus In the middle of the focus, destruction is limited to the cortex. In the front part, the softening was located under the cortical layer and reached the inner capsule. Deep-lying gray masses, corpus callosum and cingulum have survived from destruction. Microscopic examination showed extensive degeneration in connective and adhesive pathways; the descending rebirth was barely expressed and was found only in Marchi.

Differential Semiology Based on Video Electroencephalography Monitoring Between Psychogenic Nonepileptic Seizures and Temporal Lobe Epileptic Seizures.

Psychogenic nonepileptic seizures (PNESs) are disruptive changes in behavior without ictal correlate of epileptic activity and high prevalence of psychiatric morbidity. Differential diagnosis is difficult particularly with temporal lobe epilepsy (TLE), which is also associated with high prevalence of psychiatric comorbidity. Although video electroencephalography is the gold standard for differential diagnosis, clinical semiology analysis may help the clinician in general medical practice. In this study, the differential semiology, based on video electroencephalography, between PNESs and TLE seizures was analyzed. The video electroencephalography of patients with diagnosis of PNES and TLE were reviewed and compared between groups. Clinical semiology of all episodes recorded by video electroencephalography in each patient was analyzed and classified in accordance with the presence of behavioral arrest, motor hyperkinetic activity, impaired awareness, aura, and automatisms. Chi square test and binary logistic regression were determined. Thirty-two patients with PNES (32 ± 11 y) and 34 with TLE (32±12 y) were included. Female patients were predominant in the PNES group (P < 0.05). Mean time duration of episodes was 6.8±10 minutes in PNES and 1.6±0.8 minutes in TLE (P < 0.05). Impaired awareness (odds ratio=24.4; 95% confidence interval= 3.79 -157.3, P < 0.01), automatisms (odds ratio=13.9; 95% confidence interval= 2.1- 90.5, P < 0.01), and shorter duration of the events (odds ratio=2.261, 95% confidence interval= 1.149 - 4.449, P= 0.018) were found as independent factors for detecting TLE seizures comparing PNESs. Clinical semiology analysis may orientate the differential diagnosis in general medical practice, between PNESs and TLE seizures. Further studies comparing PNES semiology with other subtypes of epilepsies may complete these preliminary findings.

First Attack and Clinical Presentation of Hemiplegic Migraine in Pediatric Age: A Multicenter Retrospective Study and Literature Review.

Background: Data on clinical presentation of Hemiplegic Migraine (HM) are quite limited in the literature, particularly in the pediatric age. The aim of the present study is to describe in detail the phenotypic features at onset and during the first years of disease of sporadic (SHM) and familial (FHM) pediatric hemiplegic migraine and to review the pertinent literature.Results: Retrospective study of a cohort of children and adolescents diagnosed with hemiplegic migraine, recruited from 11 Italian specialized Juvenile Headache Centers. Forty-six cases (24 females) were collected and divided in two subgroups: 32 SHM (16 females), 14 FHM (8 females). Mean age at onset was 10.5 ± 3.8 y (range: 2–16 y). Mean duration of motor aura was 3.5 h (range: 5 min−48 h). SHM cases experienced more prolonged attacks than FHM cases, with significantly longer duration of both motor aura and of total HM attack. Sensory (65%) and basilar-type auras (63%) were frequently associated to the motor aura, without significant differences between SHM and FHM. At follow-up (mean duration 4.4 years) the mean frequency of attacks was 2.2 per year in the first year after disease onset, higher in FHM than in SHM cases (3.9 vs. 1.5 per year, respectively). A literature review retrieved seven studies, all but one were based on mixed adults and children cohorts.Conclusions: This study represents the first Italian pediatric series of HM ever reported, including both FHM and SHM patients. Our cohort highlights that in the pediatric HM has an heterogeneous clinical onset. Children present fewer non-motor auras as compared to adults and in some cases the first attack is preceded by transient neurological signs and symptoms in early childhood. In SHM cases, attacks were less frequent but more severe and prolonged, while FHM patients had less intense but more frequent attacks and a longer phase of active disease. Differently from previous studies, the majority of our cases, even with early onset and severe attacks, had a favorable clinical evolution.

Presentation of psychogenic nonepileptic seizures in Hawaii's ethnoracially diverse population

PurposeThis exploratory study compared the semiology of psychogenic nonepileptic seizures (PNES) between a diverse group of patients in the state of Hawaii. This study may expand understanding of PNES across different ethnocultural and gender groups. MethodsA retrospective chart review of patients admitted to our Epilepsy Monitoring Unit (EMU) over a 4-year period was performed to compare semiology in different ethnic groups and gender. ResultsA total of 139 patients were included in this study, 37% (n = 51) with PNES, 34% (n = 47) with epilepsy only, and 29% (n = 41) with other non-PNES, nonepilepsy diagnosis. The number of Asians with PNES were found to differ when compared with the patients with epilepsy and the patients with non-PNES, nonepilepsy diagnosis. A positive trend was found in the number of Native Hawaiians and Caucasians with PNES in comparison with patients with non-PNES, nonepilepsy diagnosis. In addition, three semiology of PNES in Native Hawaiians were found to differ in comparison with other ethnic groups with PNES: rhythmic motor, mixed semiology, and nonepileptic aura. There is a significant difference in all motor manifestation between males and females in Native Hawaiians. Between patients with PNES, patients with epilepsy, and patients with non-PNES, nonepilepsy diagnosis, significant correlation was found in psychiatric disorders including posttraumatic stress disorder (PTSD), anxiety, and any psychiatric disorder. ConclusionThis cross-cultural study found significant differences in the expression of PNES across key ethnoracial groups for the Islands of Hawaii. These findings have implications to the diagnosis and treatment of PNES for Native Hawaiians and other Pacific Islanders in the United States.

Clinical spectrum of psychogenic non epileptic seizures in children; an observational study

PurposeThe current study was designed to analyze the clinical spectrum of Psychogenic non-epileptic seizures (PNES) in children. MethodsChildren aged 6–16years with clinically suspected PNES, confirmed by short-term VEEG (STVEEG{video electroencephalogram}) and induction were classified as per Seneviratne classification. Stressors, associated co morbidities, Verbal IQ (Intelligence Quotient) and behavioral abnormalities were assessed using HTP(House tree person) test, DSM IV (Diagnostic and statistical manual of mental disorders) TR criteria, MISIC (Malin intelligence scale for Indian children) and CBCL (Child behaviour checklist). ResultsEighty children with PNES {45 boys; mean age: 10.5 (±1.6) years} were enrolled. Median delay in diagnosis was 5 months {IQR(interquartile range)- 0.5 to 48 months}) and 45% patients were already on AEDs (antiepileptic drugs). Commonest semiology was dialeptic (42.5%), followed by mixed (28.8%), motor (15%) and nonepileptic aura (13.8%). Family stressors were the commonest followed by school related issues. The most common psychiatric comorbidity was adjustment disorder. Somatic complaints were observed in 50% children. ConclusionsDialeptic PNES is commonest in children. In resource constrained settings, STVEEG along with induction is a reliable method to diagnose PNES. A comprehensive assessment protocol (including assessment of stressors) is needed for holistic management of pediatric PNES.

Sporadic Hemiplegic Migraine with SCN1A Gene Mutation—A Case Report

Sporadic hemiplegic migraine (SHM) is a subtype of hemiplegic migraine, characterized by episodes of migraine with a reversible motor aura, without a positive family history, and is a mimicker of an atypical severe form of migraine, stroke, epilepsy, multiple sclerosis, metabolic disorders, or conversion disorder.Case presentation:We present the case of a young 28-year-old female, who had a history of recurrent reversible attacks of headache with sensory aura accompanied with left hemiparesis for the past 5 years, with no positive family history of similar symptoms. The work-up ruled out differential diagnoses and genetic work-up found a novelSCN1Agene missense variation in exon 26 (c.4855A&gt;G; p.Met1619Val) in a case of SHM. She was discharged on flunarizine for prophylaxis.Conclusions:We describe, for the first time, a case of SHM with a mutation in theSCN1Agene.

Clinical profile of migraine and hemiplegic migraine in a tertiary hospital

Background: Headache is one of the most common reasons of referral in this tertiary hospital. Migraine in particular was found to affect 14% of population. However, migraine may manifest in some other form such as visual auras, sensory or motor aura, confusion, dysarthria which are poorly understood. Hemiplegic migraine as an example, needs careful assessment as this is considered to be stroke mimicker.

Olfactory hallucination in childhood primary headaches: case series.

Although olfactory hallucination (OH) has been reported in patients with primary headaches, olfactory aura has not been recognised by the International Classification of Headache Disorders (ICHD-2). In this study, we examined the frequency and characteristics of OH among children and adolescents with primary headaches. 839 neurologically normal patients with primary headaches (537 migraine) were eligible for the assessment of olfactory hallucination. Headache diagnosis was based on the ICHD. Data were prospectively collected during clinic sessions and using headache diaries. Olfactory hallucination was reported exclusively during headache attacks by 21/839 (2.5%) patients, all of whom had migraine. The prevalence of olfactory hallucination was 3.9% among migraineurs (6.5% among those with migraine aura). Olfactory hallucination shortly followed the onset of headaches and lasted from 15 to 50 minutes. Of those with MA, 10 patients had visual aura; two had somatosensory aura; one had motor aura; and two had a combination of visual and somatosensory aura. Using the ICHD-2, both OH and migraine aura occurred in the same headache attacks. In 12/15 patients, OH occurred simultaneously with migraine aura, whereas in 3/12 patients, it preceded aura. Our findings show that olfactory hallucination occurs in migraine and it has similarities to migraine aura.

Monozygotic twin sisters discordant for familial hemiplegic migraine

BackgroundThe high concordance rate of migraine in monozygotic twin pairs has long been recognised. In the current study, we present a monozygotic twin pair discordant for familial hemiplegic migraine (FHM).Case presentationsWe evaluated 12 adult family members in 2012. The twin pair was separately examined by neurologists at different time points. Mutation screening was performed for known FHM-related genes. The monozygosity of the twins was verified. Eleven individuals had a history of migraine or paroxysmal neurological symptoms, including four patients with motor aura. No mutations were detected in the CACNA1A, ATP1A2, SCN1A, PRRT2 or NOTCH3 genes. The monozygotic twin sisters, aged 52, were discordant for age of onset, motor aura and neuropsychological aura (forced thinking). Overall, the family members presented a wide range of phenotypical features.ConclusionsFamilial hemiplegic migraine is a monogenic disorder that is distinct from migraine with typical aura. However, in certain families with motor aura, such as this one, it is possible that the most severe phenotype is caused by an unlikely combination of polygenic traits and non-genetic factors. In these kindreds, we propose that hemiplegic aura is only a severe and complex form of typical aura.

Transient unilateral spatial neglect during aura in a woman with sporadic hemiplegic migraine

Hemiplegic migraine is a rare form of migraine with aura characterized by motor aura. Although auras in hemiplegic migraine are typically complex with two or more aura symptoms, neglect has been rarely described. We report the case of a 20-year-old woman with sporadic hemiplegic migraine that was investigated for the presence of unilateral spatial neglect (USN) during aura in one of her migraine attacks. Transient hemispatial neglect was observed during a right-sided migraine attack with left sensory-motor hemisyndrome; after migraine resolution there was a total recovery. Our case demonstrates that USN may be a symptom of aura. To our knowledge, this is the first report of USN during aura in an adult with sporadic hemiplegic migraine.

Génétique de la migraine

The aim of genetic studies in migraine is to identify key proteins in order to better understand the molecular mechanisms of this frequent but still incompletely understood condition. This review describes the current knowledge in the field of migraine genetics. Migraine genes have been, and still are, difficult to identify. The more common varieties of migraine are characterized by a high prevalence in the general population, and a high phenotypic variability. In the absence of any objective diagnosis marker, the status for genetic studies is established only clinically. The first breakthrough was permitted by the study of familial hemiplegic migraine, a variety of migraine with motor aura. This rare condition has a monogenic, autosomal dominant mode of inheritance, thus enabling genetic studies. The three first genes, identified from 1996 to 2005, all encode ion-channel transporters: a neuronal calcium channel (CACNA1A, FHM1), a glial sodium/potassium pump (ATP1A2, FHM2) and a neuronal sodium channel (SCN1A, FHM3). Study of cellular and animal models have shown that mutations in CACNA1A and ATP1A2 facilitated the initiation of cortical spreading depression waves, the mechanism underlying the migraine aura, and most likely increased neuronal excitability with an excess of glutamatergic neurotransmission. In 2012, PRRT2 has been identified as the fourth FHM gene, and encodes an axonal protein associated to the exocytosis complex. In the 1990s, family and twin studies showed that the more common varieties of migraine (migraine without aura and migraine with typical aura) were polygenic, with an overall heritability nearing 50 %. These genetic factors interact with environmental factors. The initial attempts to identify migraine genes by candidate gene approaches or by linkage studies were deceiving. Since 2010, three large genome-wide association studies (GWAS) have identified six genetic variants associated with migraine. Each variant has only a modest contribution to the overall genetic risk of migraine, suggesting a marked genetic heterogeneity. Three of the migraine-associated variants affect genes involved in glutamate homeostasis. Another variant concerns a gene encoding a protein implicated in nociception. Three of the four polymorphisms are associated both with migraine without aura and migraine with aura, supporting the existence of molecular mechanisms shared by all varieties of migraine. The vast majority of the migraine genes are still to be identified. Future researches will rely on new GWAS on larger cohorts of patients and controls, with a better phenotypic assessment, and on extensive sequencing.

Sporadic hemiplegic migraine with normal imaging as the initial manifestation of CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) can be present with negative family history and, especially in younger patients, with normal brain magnetic resonance. For this reason, those CADASIL patients that present only with migraine may be misdiagnosed. In the case of migraine with motor aura, sporadic hemiplegic migraine (SHM) is one of the possible misdiagnoses. We present a case of a patient who, in the first years of her disease, met the clinical criteria for SHM. A diagnosis of CADASIL was considered only when her sister presented with headache and an unknown leukoencephalopathy. This case illustrates the need for a careful review of the clinical and family history during the follow-up of primary headaches.

Management of sporadic and familial hemiplegic migraine

Sporadic and familial hemiplegic migraines (SHM and FHM) are rare paroxysmal disorders characterized by motor aura and headache. The distinction is based on whether other family members are affected. The majority of FHM families have a mutation in one of the ion channels CACNA1A, ATP1A2 and SCN1A. SHM is sometimes caused by a de novo mutation in one of the genes. Clinical trials of SHM and FHM have not been conducted. Seizure in FHM is secondary to cerebral edema and fever among other factors. The regional cerebral blood flow is reduced during the reversible aura symptoms. Triptans and ergotamine are contraindicated in the management of SHM and FHM based on the pathophysiology. Nimodipine is contraindicated. Acute and prophylactic management are otherwise based on the management principle of the migraine without aura and migraine with aura.

Is patent foramen ovale closure indicated for migraine?

Is patent foramen ovale closure indicated for migraine?

Superior oblique myokymia as a migraine motor aura

Abstract Purpose To do an epidemiological study of the proposal that superior oblique myokymia (SOM) is a migraine motor aura and propose a pathophysiological basis for the etiology of SOM. Migraine aura is a paroxysmal spreading depression of both excitatory and inhibitory neurons in the brain. These may be sensory neurons (numbness), motor neurons (dysarthria) and inhibitory neurons (chorea). Superior oblique myokymia is bursts of very rapid, jerky contrations of a superior oblique muscle causing shaking of one eye. Interfunctioning of the cortex and basal nuclei maintains stability of eye movements. Spreading depression of the cortex would cause inhibition of the inhibitory GABAergic fibres from basal ganglia to the midbrain which could result in paroxysmal firing of the 4th nerve neurons in SOM. A finely‐tuned homeostatic balance of GABAergic interneuron tone controls excitatory activation of CNS motorneurons. Small changes in GABAergic inhibition have profound effects on excitability. SOM, like auras, usually spontaneously disappears for years or permanently, which may account for the "success" of surgical treatments. The most effective treatment of SOM is gabapentin, a congener of GABA which enhances its activity. Methods Thirty seven patients with superior oblique myokymia had detailed questioning about a history of migraine headaches and auras. Results Every one of 37 patients with superior oblique myokymia had a history of migraine symptoms fulfilling all International Headache Society criteria. Every one had a history of migraine visual auras and many had also had sensory and dysphasic auras. Conclusion Epidemiological correlation and a pathophysiological explanation support the proposal that superior oblique myokymia is a migraine motor aura.