Paroxysmal Research Articles

Is there a prodrome to NMOSD? An investigation of neurologic symptoms preceding the first NMOSD attack

Background: It is unknown whether people with aquaporin-4 antibody positive (AQP4-IgG+) neuromyelitis optica spectrum disorder (NMOSD) experience a prodrome, although a few cases report AQP4 + serology up to 16 years before the first attack. Objectives: To evaluate whether individuals with AQP4-IgG + NMOSD have prodromal neurologic symptoms preceding the first attack. Methods: We reviewed medical records of participants meeting the 2015 diagnostic criteria for AQP4-IgG + NMOSD from four demyelinating disease centres in the Canadian NMOSD cohort study CANOPTICS. We searched for neurologic symptoms occurring at least 30 days before the first attack. Results: Of 116 participants with NMOSD, 17 (14.7%) had prodromal neurologic symptoms. The median age was 48 years (range 25–83) at first attack; 16 (94.1%) were female. Participants presented with numbness/tingling (n = 9), neuropathic pain (n = 5), visual disturbance (n = 4), tonic spasms (n = 2), Lhermitte sign (n = 2), severe headache (n = 2), incoordination (n = 2), weakness (n = 1), psychosis (n = 1) or seizure (n = 1). Of eight who underwent magnetic resonance imaging (MRI) brain, orbits and/or spinal cord, five had T2 lesions. Within 1.5–245 months (median 14) from the onset of prodromal neurologic symptoms, participants experienced their first NMOSD attack. Conclusions: One in seven people with NMOSD experienced neurologic symptoms before their first attack. Further investigation of a possible NMOSD prodrome is warranted.

Treatment of CACNA1A Encephalopathy and Cerebral Edema with Magnesium and Dexamethasone.

Pathogenic CACNA1A mutations can result in paroxysmal attacks of encephalopathy, hemiplegia and cerebral edema. We report two patients with CACNA1A-associated encephalopathy, hemiplegia and contralateral hemispheric cerebral edema treated successfully with intravenous magnesium sulfate and dexamethasone. One patient met the clinical criteria for familial hemiplegic migraine. There is a paucity of guidance in the literature on how to manage these patients. Despite some discrepancies in the treatment protocols in our two cases, they indicate that magnesium and dexamethasone could be part of the treatment algorithm for these patients. Further research to delineate appropriate dosing and duration of therapy is needed.

Electroclinical characteristics and therapies of tonic spasms

BackgroudEpileptic spasms followed by a tonic component have been frequently observed in patients with late-onset spasms (LOS). However, there is a lack of comprehensive analysis and summary of clinical data related to tonic spasms (TS), including seizures, video-electroencephalogram (V-EEG), synchronous electromyography (EMG) and follow-up data.MethodsTo investigate the characteristics of TS, we prospectively collected the clinical data, including 24-h V-EEG and synchronous EMG data of 32 enrolled patients who suffered from epileptic spasms followed by a TS onset at least once during the 24-h V-EEG in the epilepsy center of Xijing Hospital between June 2015 and July 2020. The patients were prescribed anti-seizure medications (ASMs) and followed up for 2–7 years.ResultsThe average age of epilepsy onset was 48.06 ± 16.07 months (range: 25 to 88 months). Among the enrolled patients, 22 patients presented with mild intellectual deficits. During the 24-h video-EEG monitoring, an average of 6.94 TS events (range: 3 to 21) were recorded, and these TS seizures often occurred in clusters. In addition to TS, 26 patients experienced generalized tonic-clonic seizures (GTCS), atypical absence seizures, myoclonic seizure, and epileptic spasms. None of the 32 patients with TS displayed hypsarrhythmia during the 24-h video-EEG recording. A total of 28 patients showed normal EEG backgrounds. Interictal epileptic discharges, including slow waves (SW), spike/sharp slow waves (SSW), and spikes, often displayed multifocally. Notably, two patients achieved seizure freedom for more than 2 years through monotherapy with oxcarbazepine (OXC), which was associated with normalization of the EEG.ConclusionsIt is difficult to classify the patients with TS as any existing epileptic syndromes, which were distinct from West syndrome or Lennox-Gastaut syndrome. TS might be an underreported seizure type and further studies are needed to gain a more comprehensive understanding of the electro-clinical features and appropriate choice of ASMs for treating tonic spasms.

A 32-year old lady who developed a complex generalized movement disorder in all four limbs after plasma exchange for myelitis

A 32-year old lady with AQP4-Antibody positive neuromyelitis optica developed a new-onset complex movement disorder after therapeutic plasma exchange, which was initially suspected to be hypocalcemic carpo-pedal spasm. However, when her bilateral, distal predominant, paroxysmal, stereotypic, wrist and finger flexor tonic contractions did not respond to serum calcium correction, other diagnoses were considered. The patient had a dramatic response to oral carbamazepine suggesting that the tonic spasms were likely a spinal movement disorder due to the primary demyelinating pathology.

Spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis: a prospective observational study

BackgroundRetrospective studies suggest that spinal movement disorders, especially tonic spasms, are prevalent in NMOSD. However, there have been no prospective studies evaluating spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis (ITM).MethodsPatients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders.ResultsSixty-three patients were evaluated from 2017 to 2021 (71% females, median age 49 years, range 18–72 years, median disease duration 12 months, range 1–408). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%). The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%). Multivariate analysis showed that longitudinally extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders, while MOG-IgG and African American race were associated with a lower risk of developing these movement disorders.ConclusionsSpinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies.

Hippocampus diffusivity abnormalities in classical trigeminal neuralgia

Abstract Introduction: Patients with chronic pain frequently report cognitive symptoms that affect memory and attention, which are functions attributed to the hippocampus. Trigeminal neuralgia (TN) is a chronic neuropathic pain disorder characterized by paroxysmal attacks of unilateral orofacial pain. Given the stereotypical nature of TN pain and lack of negative symptoms including sensory loss, TN provides a unique model to investigate the hippocampal implications of chronic pain. Recent evidence demonstrated that TN is associated with macrostructural hippocampal abnormalities indicated by reduced subfield volumes; however, there is a paucity in our understanding of hippocampal microstructural abnormalities associated with TN. Objectives: To explore diffusivity metrics within the hippocampus, along with its functional and structural subfields, in patients with TN. Methods: To examine hippocampal microstructure, we utilized diffusion tensor imaging in 31 patients with TN and 21 controls. T1-weighted magnetic resonance images were segmented into hippocampal subfields and registered into diffusion-weighted imaging space. Fractional anisotropy (FA) and mean diffusivity were extracted for hippocampal subfields and longitudinal axis segmentations. Results: Patients with TN demonstrated reduced FA in bilateral whole hippocampi and hippocampal body and contralateral subregions CA2/3 and CA4, indicating microstructural hippocampal abnormalities. Notably, patients with TN showed significant correlation between age and hippocampal FA, while controls did not exhibit this correlation. These effects were driven chiefly by female patients with TN. Conclusion: This study demonstrates that TN is associated with microstructural hippocampal abnormalities, which may precede and potentially be temporally linked to volumetric hippocampal alterations demonstrated previously. These findings provide further evidence for the role of the hippocampus in chronic pain and suggest the potential for targeted interventions to mitigate cognitive symptoms in patients with chronic pain.

A case of cerebellopontine angle meningioma presenting as neurovascular compression syndrome of the 8th cranial nerve

A 54-year-old female patient presented with paroxysmal tinnitus and vertigo for 2 years, which have repeatedly occurred while rotating her neck, and lasted about 10 seconds. An anticonvulsant medication was prescribed with a diagnosis of microvascular compression syndrome on the 8th cranial nerve and audiovestibular evaluation and magnetic resonance imaging (MRI) scan were appointed a week later. In her next visit, she reported the disappearance of paroxysmal audiovestibular symptoms after medication. In the left ear, canal paresis and abnormal auditory brainstem response were observed. In MRI, a large meningioma in the cerebellopontine angle in the vicinity of the internal auditory canal orifice was detected, that was surgically resected by a neurosurgeon. After surgical removal of the tumor, she reported continuous dizziness due to vestibular nerve injury, but the paroxysmal attack of tinnitus and vertigo disappeared without anticonvulsant medication. This case suggests that an imaging study is mandatory when diagnosing microvascular compression syndrome on the 8th cranial nerve.

Paroxysmal Ataxia: A Characteristic Feature of FGF14 Repeat Expansion (SCA27B).

Paroxysmal ataxia is typically characterized by early-onset attacks of cerebellar ataxia. Late-onset cerebellar ataxia (LOCA) comprises a group of neurodegenerative disorders mainly characterized by adult-onset progressive cerebellar ataxia. A deep intronic expansion of a GAA triplet in the FGF14 gene encoding fibroblast growth factor 14 has recently been identified as a frequent cause of LOCA. We describe a patient with paroxysmal ataxia/dysarthria due to a FGF14 repeat expansion and 3 affected family members. The 4 patients had paroxysmal ataxia/dysarthria occurring between 45 and 50 years as the initial manifestation of a FGF14 repeat expansion. The index case was investigated in detail. We have provided a video showing one of her paroxysmal episodes that could be triggered by alcohol, coffee, exertion, emotion, or cigarette smoking. Brain MRI revealed mild cerebellar atrophy, and oculography showed a subclinical downbeat nystagmus. Treatment with acetazolamide resulted in remarkable improvement. Paroxysmal dysarthria/ataxia should prompt the clinician to test for FGF14 repeat expansion/SCA27B, especially when the paroxysmal attacks are associated with late-onset cerebellar ataxia and/or a family history consistent with a dominant disorder.

Blink reflex excitability in patients with Hemifacial spasm exhibiting different abnormal discharge patterns: from early isolated discharges to later grouped bursts or tonic spasms.

We studied blink reflex (BR) and BR excitability recovery (BRER) in patients with hemifacial spasm (HFS) exhibiting different abnormal discharge patterns. We hypothesized that patients with groups of clonic or tonic burst activities appear later in the disease course and may have more excitability of the BR circuit at the brainstem compared to patients with isolated twitchings, which occur earlier. We included 124 patients with botulinum toxin-naive HFS (mean age 50.6 ± 13.3years) and 40 healthy subjects. We performed surface polymyography on facial muscles in patients and classified them according to the abnormal discharge pattern: isolated discharges, grouped bursts forming random sequences, tonic spasms, and a combination of these activities. Then, we recorded BR and BRER at 200, 600, and 1000ms interstimulus intervals. We compared disease duration, R1 and R2 latencies, R2 area-under-the-curve (AUC), and BRER% (i) between healthy subjects and patients and (ii) among groups of patients with different abnormal discharge patterns. There were isolated discharges in 28 patients, grouped bursts forming random sequences in 42, and continuous muscle activity with tonic spasms in one. The remaining patients had combinations. Mean R1 and R2 latencies were significantly longer, and mean R2 AUC was significantly higher on the symptomatic side of patients compared to healthy subjects. The mean BRER was enhanced on both sides in patients than in healthy subjects (p < 0.001). However, it was similar among patient groups with different abnormal discharge patterns (p > 0.05). The mean disease duration in patients with isolated discharges was shorter (3.3 ± 2.0years) than those with grouped bursts or tonic spasms (p = 0.002; Kruskal-Wallis test). Our study observed that excitability at the brainstem was similar in HFS patients with different abnormal discharge patterns, suggesting that the difference in discharge patterns in HFS may be due to a reason other than the difference in BR excitability.

Episodic tonic spasms in neuromyelitis optica spectrum disorder in the guise of a lancinating pain syndrome

Episodic tonic spasms in neuromyelitis optica spectrum disorder in the guise of a lancinating pain syndrome

Hypothalamic Hamartoma with Epilepsy and Psychiatric Symptoms: A Case Report

Introduction: Hypothalamic hamartomas (HH) are rare tumor malformations that can manifest as complex partial seizures refractory to anticonvulsants in adulthood and behavioral disturbances. The pathology can be misdiagnosed due to its rarity. Objectives: We attempt, from a clinical case, to alert neurologists and psychiatrists to the neurological and psychiatric symptoms of HH, knowing that they may encounter a very small number of these cases in their practice. Methods: We report the case of a 45-year-old man complaining of seizures manifested by falls, tonic spasms of the limbs and hypermotor activities. Her seizures began at the age of fifteen and presented as a deviated eye, followed by frequent seizures of varying frequency. The patient also had symptoms of intellectual disability. He was hospitalized in our psychiatry department for the management of a suicide attempt made in a hallucinatory setting, comorbid with drug-resistant epilepsy. Results: Neuroimaging concluded with the diagnosis of HH. The patient became a candidate for surgery after that. In our clinical case, the patient was able to benefit from a radio surgical treatment Gamma Knife (GK). Conclusion: In this case, the underlying etiology of the seizures and psychiatric manifestations was diagnosed thirty years after the onset of symptoms. Therefore, neurologists and psychiatrists should consider it in patients with suspicious signs and symptoms.

Spinal dystonia and other spinal movement disorders

While traditionally considered a disorder of the basal ganglia, brainstem, and cerebellum, multiple reports have shown that spinal cord pathologies may lead to dystonia. In this article, we first discuss various spinal movement disorders and the differences between tonic spasms, spinal dystonia, spinal myoclonus, spinal tremors, and paroxysmal dyskinesia. We review potential pathogenesis of spinal dystonia. We then focus on reports of dystonia secondary to spinal cord demyelinating diseases such as multiple sclerosis and neuromyelitis optica spectrum disorders. We conclude by discussing the potential treatment options for spinal dystonia.

Exome sequencing data screening to identify undiagnosed Aromatic l-amino acid decarboxylase deficiency in neurodevelopmental disorders

Aromatic l-amino acid decarboxylase (AADC) deficiency is a rare autosomal recessive neurometabolic disorder caused by biallelic pathogenic variants in the DDC gene and mainly characterized by developmental delay, hypotonia, and oculogyric crises. Early diagnosis is crucial for correct patient management; however, many patients remain misdiagnosed or undiagnosed due to the rarity and clinical heterogeneity of the disorder especially in the milder forms. Here, we applied exome sequencing approach by screening 2000 paediatric patients with neurodevelopmental disorders to identify possible new AADC variants and AADC deficiency patients. We identified five distinct DDC variants in two unrelated individuals. Patient #1 harboured two compound heterozygous DDC variants: c.436-12T > C and c.435 + 24A>C and presented with psychomotor delay, tonic spasms, and hyperreactivity. Patient #2 had three homozygous AADC variants: c.1385G > A; p.Arg462Gln, c.234C > T; p.Ala78 = , and c.201 + 37A > G and presented with developmental delay and myoclonic seizures. The variants were classified as benign class I variants and therefore non-causative according to the ACMG/AMP guidelines. Since the AADC protein is a structural and functional obligate homodimer, we evaluated the possible AADC polypeptide chain combinations in the two patients and determined the effects resulting from the amino acid substitution Arg462Gln. Our patients carrying DDC variants presented clinical manifestations not precisely overlapped to the classical symptoms exhibited by the most severe AADC deficiency cases. However, screening data derived from exome sequencing in patients featuring wide-range symptoms related to neurodevelopmental disorders may help to identify AADC deficiency patients, especially when applied to larger cohorts.

A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis.

Longitudinally extensive transverse myelitis (LETM) associated with aquaporin-4 autoantibodies (AQP4-IgG) can cause severe disability. Early diagnosis and prompt treatment are critical to prevent relapses. A novel score is described based on clinical and neuroimaging characteristics that predicts AQP4-IgG positivity in patients with LETM. Patients were enrolled both retrospectively and prospectively from multiple Italian centers. Clinical and neuroimaging characteristics of AQP4-IgG positive and negative patients were compared through univariate and multivariate analysis. Sixty-six patients were included. Twenty-seven (41%) were AQP4-IgG positive and median age at onset was 45.5 years (range 19-81, interquartile range 24). Female sex (odds ratio [OR] 17.9, 95% confidence interval [CI] 2.6-381.9; p = 0.014), tonic spasms (OR 45.6, 95% CI 3.1-2197; p = 0.017) and lesion hypointensity on T1-weighted images (OR 52.9, 95% CI 6.8-1375; p = 0.002) were independently associated with AQP4-IgG positivity. The AQP4-IgG positivity in myelitis (AIM) score predicted AQP4-IgG positivity with 85% sensitivity and 95% specificity. Positive and negative likelihood ratios were 16.6 and 0.2 respectively. The inter-rater and intra-rater agreement in the score application were both excellent. The AIM score predicts AQP4-IgG positivity with good sensitivity and specificity in patients with a first episode of LETM. The score may assist clinicians in early diagnosis and treatment of AQP4-IgG positive LETM.

Teaching NeuroImages: Ohtahara Syndrome due to Unilateral Perisylvian Polymicrogyria

A 10-month-old boy, born of nonconsanguineous parents, without any perinatal insult, presented with tonic spasms from the 13th day of life. He started having tonic seizures from 4 months of age, with global developmental delay. On examination, there were no neurocutaneous markers, facial dysmorphism, or focal neurologic deficits. EEG (figure) showed generalized and multifocal epileptiform discharges along with burst-suppression pattern. MRI of brain revealed left perisylvian polymicrogyria (figure).

Antioxidant levels in children with cyanotic breath-holding spells

Background Breath-holding spells (BHSs) are paroxysmal nonepileptic attacks that occur in some infants and toddlers. Their pathophysiology and etiology are still incompletely well defined. Aim To measure the serum levels of some antioxidant markers in children with cyanotic BHSs. Patients and methods This cross-sectional study included 50 children with cyanotic breath-holding attacks and 50 healthy control children, recruited from Pediatric Neurology Clinic and General Pediatric Clinic, Pediatric Department, Tanta University Hospital. Serum total antioxidant capacity and catalase activity were measured by the spectrophotometric method. Results Total antioxidant capacity and catalase activity levels were significantly lower in children with cyanotic BHS than in normal control children (P&lt;0.05). The number of breath-holding attacks was significantly negatively correlated with total antioxidant capacity (r=-0.77, P=0.03) and catalase activity levels (r=-0.8, P=0.04). Conclusion Children with cyanotic BHS have significantly lower levels of total antioxidant capacity and catalase activity.

PAIN MANAGEMENT IN LUNG CANCERS : EXPERIENCE OF THE MEDICAL ONCOLOGY DEPARTMENT OF HASSAN II HOSPIATL IN FEZ

Despite advances in drug therapy, pain management for patients with lung cancer remains complex. Lung cancer is known to be one of the most painful cancers. Objective: In this study we evaluate the management of pain in patients treated for lung cancer, all stages combined, in the oncology department of the Hassan II University Hospital of Fez. Mean : cross-sectional study of 71 patients collected over a period of one year using a questionnaire specifying the characteristics of the pain, its etiology, the stage of the disease, the analgesic treatments prescribed and the relief obtained by the treatment. Results: The mean age of the patients was 63.5 years with extremes ranging from 48 to 85 years. Male gender was predominant (68%), 67% of patients had metastatic cancer: 42% had bone metastases. Nociceptive pain was assessed by the visual analog scale (VAS) and neuropathic pain by the DN4 Questionnaire. Severe pain, characterized by a VAS score higher than 7, or of moderate intensity with impact on sleep and daily activity, was reported in 60.5% of our patients, mostly young subjects, all with advanced cancer. The pain was acute in 5% of cases, subacute in 32% of patients and chronic in 63% of cases. Neuropathic pain was found in 23% of patients. The pain was controlled by analgesics, level II, in association with an anti-inflammatory agent or antidepressants in 37.3%. 62.7% of patients required morphine. An average daily dose of 60mg of morphine was required to relieve pain, 15 patients reported paroxysmal pain attacks of strong intensity requiring the prescription of sublingual Fentanyl. The use of analgesic radiotherapy in combination with morphine was noted in 10 patients (9.8%). However, a small percentage (2.8%) of patients were resistant to the above-mentioned analgesics, requiring the use of non-drug techniques to relieve them. Conclusion: Our survey confirms the persistent problem of inadequate management of cancer pain. An interesting approach is to improve.

Abstract Number ‐ 234: Bilateral Non‐Painful Stimulus‐Induced Decerebrated Posturing In A Bilateral Pontine Ischemic Stroke: A Case Report

Introduction A 38 year‐old right‐handed female is a fully functional teacher with past medical history of hypertension, type II Diabetes Mellitus, polycystic ovarian syndrome, and infertility. She presented to our emergency department (ER) on the same day she was discharged twice from a local ER for acute onset persistent non‐fluctuating left‐sided weakness and painful bilateral leg spasms that woke her from sleep. Patient had pertinent exam findings of moderate pharyngeal dysarthria, mild right nasolabial flattening, mild weakness of left upper extremity (LUE), and stereotyped, painful tonic extensor posturing of the bilateral lower extremities associated with back arching and extensor response of bilateral toes. NIH Stroke Scale was 10. Methods We conducted a review of patient clinical documentation. Results MRI Brain showed anterior asymmetric bilateral pontine ischemic strokes at the ventral pontomedullary junction. MRA brain with black blood sequencing revealed multifocal vasculopathy at the right V4, mid‐basilar and left M1 segments. An angiogram showed Moya‐moya type collaterals. Tonic posturing and spasms persisted for several hours requiring muscle relaxants and benzodiazepines for symptomatic management. Unfortunately, the patient had worsening LUE weakness, inability to swallow and was found to have total vocal cord paralysis. She was intubated for airway protection and after failing extubation required tracheostomy and percutaneous gastrostomy placement. Initial workup revealed poorly controlled hyperlipidemia (LDL 162) and diabetes (A1c 8.7%), CRP 7–9, hypercoagulable panel and transthoracic echocardiogram were unremarkable. Lumbar puncture was also unremarkable (CSF TNC 2, RBC 3, glucose 116, protein 35, Enterovirus and HSV 1,2 PCR, Streptococcus antigen and VDRL were negative). Anticoagulation was started for an incidental right lower lobe subsegmental pulmonary embolism. Ultimately, the patient was discharged to inpatient rehabilitation (IPR) with a Modified Rankin Scale (mRS) of 4, on day 19th of hospitalization with some improvement in oral intake and decannulation of tracheostomy. Conclusions Movement disorders caused by strokes have been described in the literature. In their majority, they are secondary to a supratentorial injury. There have been some cases published of infratentorial, unilateral lesions that have caused different types of involuntary movements. More specifically, two papers described tonic spasms seen in pontine strokes. To our knowledge, this is a novel description of a case of bilateral pontine ischemic stroke that causes painful tonic spasms associated with decerebrate rigidity or posturing. Our hypothesis is that given the localization of the strokes in the anterior area of the inferior segment of the pons there may be involvement of the corticospinal, corticobulbar, and rubrospinal tracts which may be responsible for this presentation. Posterior circulation strokes can be missed about 9–14% of the time, possibly due to atypical presentations. We believe that presenting this case can help to improve the current available literature on this topic.

Clinical manifestations of psychogenic non-epileptic seizures in children: Experiences from a single center.

This study aimed to determine the clinical features of psychogenic non-epileptic seizures (PNES) and to enhance the accuracy of the differential diagnosis of epilepsy. This retrospective case series included patients diagnosed with PNES between December 2003 and February 2019at Taipei Veterans General Hospital. We used International Classification of Diseases (10th revision) codes for screening, and relevant medical records were reviewed. Experienced pediatric neurologists diagnosed PNES based on clinical manifestations, and occasionally on confirmatory video-electroencephalography (EEG) or simultaneous scalp-EEG during the paroxysmal attack. General information, clinical manifestations, psychological conditions, and relevant laboratory or imaging test results were collected and analyzed. Twenty-six patients (mean age, 13 years 8 months) were evaluated, 9 male and 17 female. Ten patients with PNES had a previously established diagnosis of epilepsy. The duration between symptom onset and diagnosis ranged from 1 to 120 (mean, 21; median, 12) days. Sixteen patients showed possible causative psychosocial stressors. Multiple characteristic features or specific clinical manifestations of PNES-that usually differ from epileptic seizures-were observed in all patients with PNES. A detailed evaluation of clinical manifestations and medical history is important for the accurate diagnosis of PNES.

Paroxysmal painful tonic spasms in neuromyelitis optica spectrum disorder

Recently, an association between painful tonic spasms (PTS) and Neuromyelitis Optica Spectrum Disorder (NMOSD) was established. To describe the clinical characteristics of PTS in NMOSD based on a video recording and to provide a literature review on the topic. We report a case of a 38years-old woman with a diagnosis of NMOSD and positive aquaporin-4 IgG antibody status who developed PTS five weeks after an episode of longitudinal extensive transverse myelitis (LETM). Repetitive, brief, and painful episodes of muscle contraction were observed on the patient's left hand, spreading to the left arm, and then extending to the four limbs. While pregabalin and topiramate had no influence on these episodes, the patient responded to carbamazepine (CBZ), without symptom recurrence after one year. PTS in association with LETM can be considered typical for NMOSD. Although the exact mechanism is unknown, ephaptic transmission after spinal cord damage and excitatory soluble factors released during acute inflammation responses are sought to be involved. Symptomatic treatment with CBZ achieved remission of spams in our case.