Role of a wireless surface electromyography in dystonic gait in functional movement disorders: A case report

BACKGROUND Dystonic gait(DG) is one of clinical symptoms associated with functional dystonia in the functional movement disorders(FMDs). Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation. There is no report for DG in FMDs caused by an abnormal pattern in the ankle muscle recruitment strategy during gait.CASE SUMMARY A 52-year-old male patient presented with persistent limping gait. When we requested him to do dorsiflexion and plantar flexion of his ankle in the standing and seating positions, we didn’t see any abnormality. However, we could see the DG during the gait. There were no evidences of common peroneal neuropathy and L5 radiculopathy in the electrodiagnostic study. Magnetic resonance imaging of the lumbar spine, lower leg, and brain had no definite finding. No specific finding was seen in the neurologic examination. For further evaluation, a wireless surface electromyography(EMG) was performed. During the gait, EMG amplitude of left medial and lateral gastrocnemius(GCM) muscles was larger than right medial and lateral GCM muscles. When we analyzed EMG signals for each muscle, there were EMG bursts of double-contraction in the left medial and lateral GCM muscles, while EMG analysis of right medial and lateral GCM muscles noted regular bursts of single contraction. We could find a cause of DG in FMDs.CONCLUSION We report an importance of a wireless surface EMG, in which other examination didn’t reveal the cause of DG in FMDs.

Anti-leucine-rich glioma inactivated protein 1 encephalitis with sleep disturbance as the first symptom: A case report and review of literature

BACKGROUND Anti-leucine-rich glioma inactivated protein 1(anti-LGI1) encephalitis is an infrequent type of autoimmune encephalitis(AE) characterized by acute or subacute cognitive and psychiatric disturbance, facio-brachial dystonic seizures(FBDSs), and hyponatremia. Anti-LGI1 AE has increasingly been considered a primary form of AE. Early identification and treatment of this disease are clearly very important.CASE SUMMARY Here, we report that a male patient developed severe anti-LGI1 encephalitis, which was initially misdiagnosed as a sleep disturbance. He was hospitalized for epileptic seizures and typical FBDSs half a month after he developed sleep disturbances. LGI1 antibodies were detected in his cerebrospinal fluid and serum(1:100 and 1:3.2, respectively), which led to the diagnosis of classic anti-LGI1 AE. No obvious abnormality was observed on brain computed tomography images. T2-weighted fluid-attenuated inversion recovery and T2-weighted scans of brain magnetic resonance imaging(MRI) showed slightly elevated signals within the left basal ganglia area. No tumor was detected within the brain of this patient using MRI. After hormone and antiepileptic drug treatment, the patient’s symptoms improved significantly.CONCLUSION Anti-LGI1 antibody-associated encephalitis has characteristic clinical manifestations, such as cognitive impairment, psychiatric symptoms, seizures, sleep disorders, hyponatremia, and FBDSs. LGI1 antibodies are present in the serum and/or cerebrospinal fluid, but their production is sensitive to immunosuppressants, and this disease has a relatively good prognosis. In particular, we should be aware of the possibility of anti-LGI1 antibody-associated encephalitis in adolescents with sleep disorders to avoid missed diagnoses and misdiagnoses.

Sandifer’s Syndrome in a 3-Month-Old Male Infant: A Case Report

Background: Sandifer syndrome (SS) is the association of gastroesophageal reflux disease (GERD) with neurological manifestations (spastic torticollis and paroxysmal dystonic postures with arching of the back and rigid opisthotonic posturing. Case presentation: A 3-month-old male infant presented to our emergency department with torticollis and dystonic episodes for two months associated with vomiting. These movements were observed during or just after feeding. Since the patient developed regurgitations with torticollis and dystonic episodes with arching of the back and rigid opisthotonic posturing. The electroencephalogram was normal. Barium swallow/meal examination revealed GERD without evidence of hiatus hernia. Apgar scores were 7 at 1 min and 9 at 5 min. Conclusion: Early recognition and treatment of GERD in patients with Sandifer syndrome enhance the success of medical management and contributes to improved quality of life for patients with brain damage. The paroxysmal dystonic behaviors were dramatically disappeared completely after medical management in this patient.

Heterogeneity of clinical features, EEG and brain imaging findings in anti‑leucine‑rich glioma‑inactivated protein 1 autoimmune encephalitis: a retrospective case series study and review of the literature

Background Anti-leucine-rich glioma-inactivated 1(LGI-1)autoimmune encephalitis(AE),characterized by rapid decline of memory,seizures,and neuropsychiatric abnormalities,is a rare but devastating disorder.Early diagnosis and treatment are essential to prevent long-term sequelae.In this report,we provide a detailed description of clinical characteristics,laboratory test results,imaging,and electroencephalography(EEG)findings,as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center.Case presentation At the onset,all eight patients presented with confusion/memory deterioration,seizures(including faciobrachial dystonic seizures or other types of seizure),and behavioral changes such as hallucination,paranoia,and anxiety.Four patients were found with severe hyponatremia.Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients.For patients with faciobrachial dystonic seizures,no discernible scalp EEG change was detected,while EEG recording of patients experiencing other types of seizure showed focal slowing,focal epileptiform discharges,and focal onset seizures.All patients showed abnormal brain magnetic resonance imaging signals,mainly involving the mesial temporal lobe and the hippocampus.In addition,one patient also experienced fulminant cerebral edema during the acute phase of the illness.All patients received immunotherapy and anti-seizure medications and achieved good seizure control.Nevertheless,these patients continued to experience cognitive impairment during their long-term follow-ups.Conclusions The care of anti-LGI1 AE patients requires rapid evaluation,prompt initiation of immunotherapy,and long-term follow-up.The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features.Further research is needed to understand the molecular mechanisms underlying the heterogeneity,in order to facilitate development of more effective treatments for anti-LGI1 AE.

Neurological diseases associated with autoantibodies targeting the voltage-gated potassium channel complex:immunobiology and clinical characteristics

Voltage-gated potassium channels(VGKCs)represent a group of tetrameric signaling proteins with several functions,including modulation of neuronal excitability and neurotransmitter release.Moreover,VGKCs give a key contribution to the generation of the action potential.VGKCs are complexed with other neuronal proteins,and it is now widely known that serum autoantibodies directed against VGKCs are actually directed against the potassium channel subunits only in a minority of patients.By contrast,these autoantibodies more commonly target three proteins that are complexed with alpha-dendrotoxin-labeled potassium channels in brain extracts.These three proteins are contactin-associated protein-2(Caspr-2),leucine-rich,glioma inactivated 1(LGI-1)protein and the protein Tag-1/contactin-2.Neoplasms are detected only in a minority of seropositive patients for VGKC complex-IgG and do not significantly associate with Caspr-2 or LGI-1.Among all the cancers described in association with VGKC complex-IgG,lung carcinoma,thymoma,and hematologic malignancies are the most commonly detected.We will review all the major neurological conditions associated with VGKC complex-IgG.These include Isaacs’syndrome,Morvan syndrome,limbic encephalitis,facio-brachial dystonic seizures,chorea and other movement disorders,epilepsy,psychosis,gastrointestinal neuromuscular diseases,a subacute encephalopathy that mimics Creutzfeldt-Jakob prion disease both clinically and radiologically and autoimmune chronic pain.The vast majority of these conditions are reversible by immunotherapy,and it is becoming increasingly recognized that early diagnosis and detection of VGKC complex-IgG is critical in order to rapidly start the treatment.As a result,VGKC complex-IgG are now part of the investigation of patients with unexplained subacute onset of epilepsy,memory or cognitive problems,or peripheral nerve hyperexcitability syndromes.