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Background: Subacute sclerosing panencephalitis (SSPE) is a complication of measles, occurring after a latency of 4-10 years. It continues to occur in developing countries although resurgence is being reported from developed countries. Characteristic features include progressive neuropsychiatric issues, myoclonus, seizures, movement disorders and visual impairment. Electroencephalography (EEG) typically shows periodic generalized discharges, and elevated CSF anti-measles antibodies are diagnostic. Movement disorders are being increasingly recognized as part of the clinical spectrum, and range from hyperkinetic (chorea, dystonia, tremor, tics) to hypokinetic (parkinsonism) disorders and ataxia., Objectives: This article aims to comprehensively review the spectrum of movement disorders associated with SSPE., Methods: A literature search was conducted in PubMed and EMBASE databases in December 2023 and articles were identified for review., Results: Movement disorders reported in SSPE included hyperkinetic (chorea, dystonia, tremor and tics), hypokinetic (parkinsonism), ataxia and extraocular movement disorders. Myoclonus, a core clinical feature, was the most frequent "abnormal movement." Movement disorders were observed in all clinical stages, and could also be a presenting feature, even sans myoclonus. Hyperkinetic movement disorders were more common than hypokinetic movement disorders. An evolution of movement disorders was observed, with ataxia, chorea and dystonia occurring earlier, and parkinsonism later in the disease. Neuroradiological correlates of movement disorders remained unclear., Conclusion: A wide spectrum of movement disorders was observed throughout the clinical stages of SSPE. Most data were derived from case reports and small case series. Multicentric longitudinal studies are required to better delineate the spectrum and evolution of movement disorders in SSPE., (© 2024 International Parkinson and Movement Disorder Society.)

Background: Post-stroke movement disorders (PSMD) encompass a wide array of presentations, which vary in mode of onset, phenomenology, response to treatment, and natural history. There are no evidence-based guidelines on the diagnosis and treatment of PSMD., Objectives: To survey current opinions and practices on the diagnosis and treatment of PSMD., Methods: A survey was developed by the PSMD Study Group, commissioned by the International Parkinson's and Movement Disorders Society (MDS). The survey, distributed to all members, yielded a total of 529 responses, 395 (74.7%) of which came from clinicians with experience with PSMD., Results: Parkinsonism (68%), hemiballismus/hemichorea (61%), tremor (58%), and dystonia (54%) were by far the most commonly endorsed presentation of PSMD, although this varied by region. Basal ganglia stroke (76% of responders), symptoms contralateral to stroke (75%), and a temporal relationship (59%) were considered important factors for the diagnosis of PSMD. Oral medication use depended on the phenomenology of the PSMD. Almost 50% of respondents considered deep brain stimulation and ablative surgeries as options for treatment. The lack of guidelines for the diagnosis and treatment was considered the most important gap to address., Conclusions: Regionally varying opinions and practices on PSMD highlight gaps in (and mistranslation of) epidemiologic and therapeutic knowledge. Multicenter registries and prospective community-based studies are needed for the creation of evidence-based guidelines to inform the diagnosis and treatment of patients with PSMD., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: The human immunodeficiency virus (HIV) causes movement disorders in persons living with HIV (PLH)., Objectives and Methods: We conducted a systematic review on the spectrum of movement disorders in PLH using standard terms for each of the phenomenologies and HIV., Results: Movement disorders in PLH were commonly attributed to opportunistic infections (OI), dopamine receptor blockade reactions, HIV-associated dementia (HAD), presented during seroconversion, developed due to drug reactions or antiretroviral therapy (ART) itself and lastly, movement disorders occurred as a consequence of the HIV-virus. Parkinsonism in ART naïve PLH was associated with shorter survival, however when Parkinsonism presented in PLH on ART, the syndrome was indistinguishable from Idiopathic Parkinson's disease and responded to therapy. Tremor was often postural due to HAD, drugs or OI. Generalized chorea was most frequent in HIV encephalopathy and toxoplasmosis gondii caused most cases of hemichorea. Ataxia was strongly associated with JCV infection, ART efavirenz toxicity or due to HIV itself. Dystonia was reported in HAD, secondary to drugs and atypical facial dystonias. Both cortical/subcortical and segmental/spinal origin myoclonus were noted mainly associated with HAD. In patients with HIV related opsoclonus-myoclonus-ataxia-syndrome, seroconversion illness was the commonest cause of followed by IRIS and CSF HIV viral escape phenomenon., Conclusions: Aetiology of movement disorders in PLH depend on the treatment state. Untreated, PLH are prone to develop OI and HAD and movement disorders. However, as the number of PLH on ART increase and survive longer, the frequency of ART and non-AIDS related complications are likely to increase., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest relevant to this work., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Drug-induced movement disorders (DIMDs) are most commonly associated with typical and atypical antipsychotics. However, other drugs such as antidepressants, antihistamines, antiepileptics, antiarrhythmics, and gastrointestinal drugs can also cause abnormal involuntary movements. Different types of movement disorders can also occur because of adverse drug reactions. Therefore, the important key to diagnosing DIMDs is a causal relationship between potential offending drugs and the occurrence of abnormal movements. The pathophysiology of DIMDs is not clearly understood; however, many cases of DIMDs are thought to exert adverse mechanisms of action in the basal ganglia. The treatment of some DIMDs is quite challenging, and removing the offending drugs may not be possible in some conditions such as withdrawing antipsychotics in the patient with partially or uncontrollable neuropsychiatric conditions. Future research is needed to understand the mechanism of DIMDs and the development of drugs with better side-effect profiles. This article reviews the phenomenology, diagnostic criteria, pathophysiology, and management of DIMDs., Competing Interests: None declared., (Thieme. All rights reserved.)

Poststroke movement disorders (PSMDs) are a common cause of secondary movement disorders. Although prior studies have highlighted the clinical spectrum and phenomenology of PSMDs, there are many knowledge gaps worth addressing. Some of the most important include lack of clinical definitions, variable stroke symptom latencies, and lack of biomarkers for vulnerability for or resilience against developing PSMDs. Collectively, the association between stroke localization and phenomenology is less than 30%, and the long-term clinical prognosis and treatment responses are highly variable. After summarizing the accumulated evidence regarding the phenomenology, pathophysiology, neuroimaging, and treatment of PSMDs, highlighting the many gaps and controversies including diagnostic challenges, we propose a roadmap for future research to fill these gaps and resolve the related controversies. More research is warranted concerning the phenomenology, classification, diagnostic criteria, and pathophysiology of PSMDs. Further, there is an urgent need for treatment guidelines for the management of PSMDs. © 2022 International Parkinson and Movement Disorder Society., (© 2022 International Parkinson and Movement Disorder Society.)

Objectives: To detail the spectrum of movement disorders (MD) among children with cerebral palsy (CP) and assess impact on functional status., Methods: In this cross-sectional study, children with CP were recruited and examined for various MDs. Tone abnormality was assessed using Hypertonia Assessment Tool (HAT), functional status using Gross Motor Function Classification System Expanded and Revised (GMFCS E&R), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS). These scores were classified into mild-moderate (level I-III)/severe (level IV-V) categories., Results: A total of 113 children (mean age 4.9 ± 3.4 y, 66.4% boys) were enrolled. MDs were noted in 52 (46%) children; the most frequent were dystonia (28%), chorea (14%), choreoathetosis (8%). Of 64 children with quadriparetic CP, 27 (42.2%) demonstrated MDs. Of 19 children with hemiparetic CP, 2 (10.5%) had MDs. Of 16 children with dyskinetic CP, 15 (93%) had MDs. Children with dyskinetic CP had significantly higher frequency of MDs (p = 0.001). There was no difference in occurrence of all MDs or dystonia aloneamongst the two categories (mild-moderate/severe) of GMFCS E&R levels, CFCS levels or MACS levels., Conclusion: Although diverse MDs occur frequently in CP, these do not correlate with the broad functional status of the child. The study is limited by small sample size., (© 2021. Dr. K C Chaudhuri Foundation.)

Context: Movement disorders represent a common presentation in pediatrics and are often a source of clinical and diagnostic dilemmas. In this review, we provide an overview of common causes along with simplified clinical approach and management options for major movement disorders., Sources: This narrative review is based on contemporary evidence and personal experience. Medline was searched for recent advances, current understanding and consensus on classification, clinical features, diagnosis and treatment., Results: Movement disorders are classified as hyperkinetic and hypokinetic disorders, the latter being rare in childhood. The hyperkinetic disorders include dystonia, chorea, athetosis, tics and tremor, stereotypies, myoclonus, startle syndromes and functional disorders. Some movement disorders can be benign and developmental. A large proportion of conditions are genetic in origin with a guarded prognosis. Some of the conditions may be post-infectious, immune-mediated or drug induced. Multiple types of movement disorders are present in many conditions. The age at onset, type and distribution of abnormal movements and presence of associated neurological and systemic features help in narrowing the differential diagnosis. The pharmacotherapy of movement disorders is complex and evolving., Conclusion: A synopsis of movement disorders presenting in pediatric age has been provided, incorporating the latest evidence. A simplified approach for clinical diagnosis has been developed for dystonia and chorea.

Involuntary movements develop after 1-4% of strokes and they have been reported in patients with ischemic and hemorrhagic strokes affecting the basal ganglia, thalamus, and/or their connections. Hemichorea-hemiballism is the most common movement disorder following a stroke in adults while dystonia is most common in children. Tremor, myoclonus, asterixis, stereotypies, and vascular parkinsonism are other movement disorders seen following stroke. Some of them occur immediately after acute stroke, some can develop later, and others may have delayed onset progressive course. Proposed pathophysiological mechanisms include neuronal plasticity, functional diaschisis, and age-related differences in brain metabolism. There are no guidelines regarding the management of post-stroke movement disorders, mainly because of their heterogeneity., Competing Interests: None

Background: Repetitive behaviors refer to a broad class of responses ranging from stereotypic body movements to impulsive/compulsive behaviors. They may be associated with neurological disorders., Methods: This is a case series of six subacute sclerosing panencephalitis (SSPE) patients who presented with a wide spectrum of repetitive motor behaviors and vocalizations., Results: Repetitive motor behaviors involved the upper limbs in all patients and lower limbs in 3 patients. The repetitive movements in the upper limbs were clapping, finger-clicking, hand rubbing, flailing, and dystonic posturing. In the lower limbs, the repetitive movements were rubbing with the heel, pelvic thrusting with flexion extension of the leg, and foot tapping. The spectrum of vocalizations included palilalia, whistling, grunting with spitting, and pathological crying. Repetitive behaviors were the presenting features in 2 patients., Conclusions: This case series expands the spectrum of repetitive behaviors seen in neurological disorders associated with brain infections. © 2020 International Parkinson and Movement Disorder Society., (© 2020 International Parkinson and Movement Disorder Society.)

Aim of Study: Botulinum neurotoxin type A (BoNT/A) injections are the established treatment in cervical dystonia (CD). But clinical practice regarding the choice of muscles into which injections are made varies between centres. Until now, there have been no dose-per-muscle recommendations based on 'searching the dose' clinical trial data., Clinical Rationale for Study: We therefore examined the dosages under real world conditions at seven international movement disorders centres, using an identical clinical approach., Results: We examined 305 patients with CD (55.6 ± 13.2 years, 204 female). The most commonly injected muscles were the splenius capitis (84.9%), sternocleidomastoid (80.3%), trapezius (59.7%), levator scapulae (49.8%), semispinalis capitis (39%), and obliquus capitis inferior (36.7%). The mean total dose per treatment session with aboBoNT/A was 652.5 (SD = 285.5), with onaBoNT/A it was 159.5 (SD = 62.4), and with incoBoNT/A it was 173.4 (SD = 99.2) units. The doses injected into each muscle in the ona- or incoBoNT/A groups were between 19.7 and 48.2 units, with the highest dose for the splenius capitis with 49.2 ± 26.0 units. The doses in the aboBoNT/A group were between 69.6 and 146.4 units, and the highest dose being injected into the splenius capitis (139.6 ± 80.7 units)., Conclusions and Clinical Implications: In clinical trials the doses per muscle are based on an arbitrary decision. In our study, the doses were lower than in other studies, which may be due to the number of muscles per session, the use of ultrasound guidance (and therefore more precise injections), as well as the use of the Col-Cap concept. Our results exemplify everyday practice, and may help as the basis for recommendations and further investigations.

Autoimmune encephalitis (AIE) constitutes an important treatable cause of movement disorders. We aimed to highlight the spectrum of movement disorder and other salient features of AIE patients diagnosed at our tertiary care centre and describe their clinical symptoms, diagnostic approach, treatment, and outcome. We evaluated 11 patients who presented with movement disorder in association with AIE at our centre. Various abnormal movements observed were tremor, dyskinesias, stereotypy, dystonia, ataxia, asterixis, myoclonus, and parkinsonism. Antibodies were detected against NMDAR (n = 3), LGI-1 (n = 2), GAD-65 (n = 1), CASPR-2 (n = 1), Sox-1 (n = 1), Yo (n = 1), and thyroid peroxidase (n = 1). One patient was diagnosed with opsoclonus myoclonus syndrome associated with the suspected neuroblastic tumour. Six patients responded well to first-line immunotherapy (intravenous immunoglobulins or steroid or both). Three patients with anti-NMDAR antibodies received second-line therapy consisting of rituximab. Movement disorder is one of the most consistent features of AIE. Understanding of the ever-expanding spectrum of antibodies associated with movement disorders helps in the early diagnosis and better management of patients of autoimmune movement disorder., (© 2021 S. Karger AG, Basel.)

Competing Interests: The authors have no competing interests to declare.

Background: Cranial functional movement disorders (CFMDs) affect the face, eyes, jaw, tongue, and palate., Objectives: We aimed to examine our large series of functional movement disorders (FMDs) patients where the cranial muscles were involved to determine their phenomenology and other clinical features., Methods: This is a chart review of 26 patients who presented with CFMDs., Results: There were 16 (61.53%) females and 10 (38.46%) male patients. The mean ± [standard deviation (SD)] age at the presentation was 33.96 ± 16.94 (Range: 11-83) years. The duration of symptoms ranged from one day to 6 years (Mean ±SD: 402.03 ±534.97 days). According to the Fahn-Williams criteria, CFMDs were documented in 24 patients and clinically established in two patients. The facial [38.46% (10/26)] involvement was the most common in our CFMDs patients. Oromandibular [19.23% (5/26)], ocular [15.38% (4/26)], lingual [15.38% (4/26)], speech [15.38% (4/26)] and palatal [(3.85; 1/26)] involvement was also seen. 10 (38.46%) patients also had associated FMD in the extracranial regions. Precipitating factors were present in 84.61% (22/26) of the patients and associated illnesses were present in 42.30% (11/26) of the patients. At 3 months follow-up, 9 (34.61%) patients had improved, 13 (50%) had partial improvement and 4 (15.38%) had no improvement., Conclusions: There was a slight female preponderance in our patients. CFMDs are more likely to involve facial muscles. Associated medical conditions like neuropsychiatric disturbances and headaches are frequently present in CFMDs patients. Early clinical diagnosis will avoid unnecessary investigations and allow the patient to seek the right treatment., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2020 The Author(s).)

Background: Functional movement disorders (FMDs) have been rarely described in the elderly population., Methods: This is a retrospective chart review of elderly patients with FMDs (onset >60 years) attending the movement disorders clinic at a tertiary care teaching institute in India., Results: Out of 117 patients diagnosed with FMD at our center, 18 patients had an onset after the age of 60 years. The male-to-female ratio was 10:8 and the duration of symptoms ranged from 1 day to 5 years. Social (10/18) and physical factors (5/18) with an evident temporal relationship with the onset of FMD were identified in 15 out of 18 patients. Six of them had a past history of depression, anxiety, or other psychiatric illnesses. The tremor was the most frequent phenomenology seen in 11 (61.1%) patients, followed by dystonia in seven (38.8%), choreoballism and tics in two each, and hemifacial spasm and functional gait in one each. Seven patients had more than one phenotype., Discussion: Tremor was the most frequent movement disorder seen in our patients with FMD. Surprisingly, tics ( n = 2) and choreoballistic ( n = 2) movements were also found in our patients with FMD, which has not been reported previously in an elderly population. Both physical and social factors were identified preceding the development of FMDs in majority of our patients., Competing Interests: Funding: None. Conflicts of Interest: The authors report no conflicts of interest. Ethics Statement: This study was performed in accordance with the ethical standards detailed in the Declaration of Helsinki. This study was a retrospective chart review and all patients had provided written informed consent.

Movement disorders are one of the common clinical features of neurological disease associated with neuronal antibodies which is a group of potentially reversible disorder. They can present with hypokinetic or hyperkinetic types of involuntary movements and may have other associated neurological symptoms. The spectrum of abnormal movements associated with neuronal antibodies is widening. Some specific phenomenology of movement disorders are likely to give clue about the type of antibody, for instance, presence of paroxysmal dystonia (facio-brachial dystonic seizures) are a pointer toward presence of LGI-1 antibodies, and orofacial lingual dyskinesia is associated with NMDAR associated encephalitis. The presence of specific type of movement disorder allows high suspicion of testing of certain specific type of antibodies. In this review, we have discussed the various antibodies and the spectrum of movement disorder associated with them, highlighting if any distinct movement disorder allows the clinician to suspect type of antibody in a certain clinical context. We have also reviewed the treatment of the movement disorder associated with the neuronal antibodies. Physicians should have high index of suspicion of these disorders, as early institution of treatment options can lead to better outcome., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Botulinum toxin has gained immense popularity since its introduction for therapeutic use. It is used in a variety of movement disorders like hemi-facial spasm, focal dystonias like blepharospasm, cervical dystonia, oromandibular dystonia, limb dystonias. It is also being used in patients with tremors, tics and for a variety of indications in Parkinson's disease as well. There are eight subtypes of toxins available, but type A and B are the ones used in movement disorder clinics. The toxin mainly acts by inhibiting the release of acetylcholine at the neuromuscular junction and causing weakness. Type B toxin has more effect over the autonomic nervous system and hence is preferred for hyper-secretory disorders. The use of electromyography and ultrasound further improve the accuracy of the procedure. It is a relatively safe therapeutic option with its effect lasting for around three months. It has very few side effects. The key is to start with the lowest possible dose and then gradually increase the dose depending upon the patient's response. Selecting the right muscles for injection is of utmost importance and is guided by the knowledge of anatomy of the muscles., Competing Interests: There are no conflicts of interest.

Background: After a stroke, movement disorders are rare manifestations mainly affecting the deep structures of the brain like the basal ganglia (44%) and thalamus (37%), although there have been case studies of movement disorders in strokes affecting the cerebral cortex also., Summary: This review aims to delineate the various movement disorders seen in association with thalamic strokes and tries to identify the location of the nuclei affected in each of the described movement disorders. Cases were identified through a search of PubMed database using different search terms related to post-thalamic stroke movement disorders and a secondary search of references of identified articles. We reviewed 2,520 research articles and only 86 papers met the inclusion criteria. Cases were included if they met criteria for post-thalamic stroke movement disorders. Case-cohort studies were also reviewed and will be discussed further. Key Messages: The most common post-stroke abnormal movement disorder reported in our review was dystonia followed by hemiataxia. There was a higher association between ischaemic stroke and movement disorder. Acute onset movement disorders were more common than delayed. The posterolateral thalamus was most commonly involved in post-thalamic stroke movement disorders., (© 2018 S. Karger AG, Basel.)

Context: Painful leg and moving toes (PLMT) syndrome is a rare movement disorder where the patient has pain followed by movement disorder in one or both lower limbs. The exact etiology and pathogenesis is uncertain, however many cases have been related to lesions in peripheral nerve, spinal cord or radicals. Appearance of abnormal movement in PLMT soon after surgery has not, to our knowledge, been described., Findings: We report a 40-year-old female patient who had secondary tethered cord syndrome. She had pain in left lower limb for 5 months prior to surgery. Immediately after surgery, she noticed abnormal movement in left foot. Surface electromyography suggested single motor unit discharges., Conclusion/clinical Relevance: In persons with unclear symptoms of painful leg and moving toes, PLMT syndrome should be in the differential diagnosis.

Deep brain stimulation (DBS) is a major advancement in the field of functional neurosurgery in the last century. This treatment option is now utilized for many hyperkinetic and hypokinetic movement disorders and certain disorders in the field of psychiatry. The basis of treatment is a by-product of excellent advancement made in the field of basic neuroscience and technology. Due to the improvement in the field of neuroimaging, brain structures are now better localized leading to a better outcome. Newer sites of stimulations are being recognized, which may further improve the clinical outcome in patients. However, it is very important to stick to stringent inclusion and exclusion criteria while selecting patients for DBS to get the best results.

Background: Functional movement disorders (FMDs) and functional seizure (FS) are the two most important subcategories of functional neurologic disorders (FNDs). Objectives: This study aimed to discern similarities and differences between patients with FMD and FS. Methods: A prospective comparative study of 94 patients with FNDs (FMD = 47, FS = 47) was conducted. Results: Tremor and pauci-kinetic attack with preserved responsiveness were the most common subtypes observed in patients with FMD and FS, respectively. A significantly higher number of patients with FMD had more than one precipitating factor (P = 0.03). Headache was significantly more common in patients with FS (P = 0.03). More patients came for follow-up in the FMD group (P = 0.01). More patients in the FS group reported "very much improvement" (P = 0.04), and "no change" was more commonly reported by the FMD group patients (P = 0.009). Conclusion: Emotional stress was the most common precipitating factor in patients with FMD and FS. The prognosis was better in patients with FS. [ABSTRACT FROM AUTHOR]

Byline: . Karanvir, Divyani. Garg, Suvasini. Sharma, Sanjay. Pandey Sir, Infantile tremor syndrome (ITS) is a well-characterized neuro-cutaneous syndrome caused by vitamin B12 deficiency, occurring in children between 5 months [...]

BACKGROUND AND AIM: Clinical services were severely affected globally during the COVID-19 pandemic. This study aimed to characterize the clinical experience of using botulinum toxin (BTX) injections during the COVID-19 pandemic. METHODS: This is a retrospective chart review of patients who received BTX injections from April 2019 to January 2022. RESULTS: A total of 105 patients received an BTX injections, out of which 76 (72.4%) were men. The mean age of the patients was 47.9±15.1 years. The most common indication for receiving BTX injections was dystonia (n = 79; 75.2%), followed by hemifacial spasm (n = 22; 21%) and miscellaneous movement disorders (n = 4; 3.8%). Focal dystonia (n = 45; 57%) was the most frequent form of dystonia, followed by segmental dystonia (n = 24; 30%). The percentage of generalized dystonia and hemidystonia was 12% and 1%, respectively. Cervical dystonia (44.4%), blepharospasm (17.8%), and writer’s cramp (15.6%) were the most frequent forms of focal dystonia. The miscellaneous group included four patients (3.8%) with trigeminal neuralgia, Holmes tremor, dystonic tics, and hemimasticatory spasm. The mean ages of patients in the dystonia, hemifacial spasm, and the miscellaneous groups were 47.7±14.9 years, 49.2±14.0 years, and 44.2±26.0 years, respectively. The mean BTX dose was 131.6±104.1 U. The mean BTX doses for the dystonia group, hemifacial spasm, and the miscellaneous group were 158.7±105.3 U, 40.1±11.3 U, and 100.0±70.7 U, respectively. CONCLUSION: Most patients in our cohort had dystonia, followed by hemifacial spasm. Among the patients with dystonia, most had focal dystonia, with cervical dystonia being the most common movement disorder. The data obtained in our study is important to increase awareness of the effectiveness of BTX injections in patients with chronic disorders. [ABSTRACT FROM AUTHOR]

Background: Hypermanganesemia with dystonia 1 and 2 (HMNDYT1 and 2) are rare, inherited disorders of manganese transport. Objectives: We aimed to describe clinical, laboratory features, and outcomes among children with HMNDYT. Methods: We conducted a retrospective multicenter study involving tertiary centers across India. We enrolled children between 1 month to 18 years of age with genetically confirmed/clinically probable HMNDYT. Clinical, laboratory profile, genetic testing, treatment details, and outcomes scored by treating physicians on a Likert scale were recorded. Results: We enrolled 27 children (19 girls). Fourteen harbored SLC30A10 mutations; nine had SLC39A14 mutations. The SLC39A14 cohort had lower median age at onset (1.3 [interquartile range (IQR), 0.7–5.5] years) versus SLC30A10 cohort (2.0 [IQR, 1.5–5.1] years). The most frequent neurological features were dystonia (100%; n = 27), gait abnormality (77.7%; n = 21), falls (66.7%; n = 18), and parkinsonism (59.3%; n = 16). Median serum manganese (Mn) levels among SLC39A14 (44.9 [IQR, 27.3–147.7] mcg/L) cohort were higher than SLC30A10 (29.4 [17.1–42.0] mcg/L); median hemoglobin was higher in SLC30A10 (16.3 [IQR, 15.2–17.5] g/dL) versus SLC39A14 cohort (12.5 [8.8–13.2] g/dL). Hepatic involvement and polycythaemia were observed exclusively in SLC30A10 variants. A total of 26/27 children underwent chelation with disodium calcium edetate. Nine demonstrated some improvement, three stabilized, two had marked improvement, and one had normalization. Children with SLC39A14 mutations had poorer response. Two children died and nine were lost to follow‐up. Conclusions: We found female predominance. Children with SLC39A14 mutations presented at younger age and responded less favorably to chelation compared to SLC30A10 mutations. There is emerging need to better define management strategies, especially in low resource settings. [ABSTRACT FROM AUTHOR]

Movement disorders may be one of the neurological manifestations of demyelinating disorders. They can manifest in Parkinsonism or a wide spectrum of hyperkinetic movement disorders including tremor, paroxysmal dyskinesia, dystonia, chorea, and ballism. Some of these disorders occur during an acute episode of demyelination, whereas others can develop later or even may precede the onset of the demyelinating disorders. The pathophysiology of movement disorders in demyelination is complex and the current evidence indicates a wide involvement of different brain networks and spinal cord. Treatment is mainly symptomatic and oral pharmacological agents are the mainstay of the management. Botulinum toxin and neurosurgical interventions may be required in selected patients. [ABSTRACT FROM AUTHOR]

The association of movement disorders (MDs) with musculoskeletal (MSK) disorders is observed in two principal scenarios. First, MDs patients may present with MSK issues. This phenomenon is primarily observed in parkinsonian syndromes, but may also be seen in patients with dystonia, Tourette syndrome, and some gene‐related MDs. Second, there are MSK disorders that may produce or mimic MDs. Important primary MSK disorders producing MDs are joint hyperlaxity syndrome, non‐traumatic craniovertebral junction anomalies, congenital muscular torticollis, and rheumatoid arthritis. Peripheral trauma to the MSK system may also lead to MDs commonly referred to as peripherally induced MDs. The exact pathogenesis of these disorders is not clear, however many patients have associated sensory phenomena such as complex regional pain syndrome. Herein, we provide an overview of disorders that may manifest with a combination of MSK and MDs, as detailed above. The most common MDs are discussed in each section, along with important clinical points, suggested diagnostic workups, and possible differential diagnoses. [ABSTRACT FROM AUTHOR]

Functional movement disorders (FMDs) are not uncommon in children. The age at onset may have a bearing on the phenomenological pattern of abnormal movement, risk factors, and response to different treatment modalities in this age group. FMDs in children resemble their adult counterparts in terms of gender preponderance, but risk factors are quite different, and often influenced by cultural and demographic background. FMDs contribute to a significant proportion of acute pediatric movement disorder patients seen in emergency settings, ranging from 4.3 to 23% in different case series. The most common movement phenomenologies observed in pediatric FMDs patients are tremor, dystonia, gait disturbances, and functional tics. Various social, physical, and familial precipitating factors have been described. Common social risk factors include divorce of parents, sexual abuse, bullying at school, examination pressure, or other education-related issues, death of a close friend, relative, or family members. Physical trauma like minor head injury, immunization, tooth extraction, and tonsillectomy are also known to precipitate FMDs. The response to treatment appears to be better among pediatric patients. We aim to review FMDs in children to better understand the different aspects of their frequency, clinical features, precipitating factors, diagnosis, treatment, and outcome. [ABSTRACT FROM AUTHOR]

Insulinoma-Induced Hypoglycemia with Generalized Chorea, Dystonia, and Ataxia: A Neurological Kaleidoscope Keywords: Insulinoma; hypoglycemia; chorea EN Insulinoma hypoglycemia chorea S6 S8 3 09/06/21 20210802 NES 210802 It is well acknowledged that a plethora of neuropsychiatric manifestations such as headache, confusion, visual disturbances, seizures, dysarthria, a stroke-like picture, ataxia, motor neuropathy or cramps, and paroxysmal choreoathetosis can occur in patients with an insulinoma attributed to the various ways neuroglycopenia tends to affect different parts of the brain, and if not recognized or if misinterpreted, may result in permanent neurological damage or even death.1-3 We report a patient who presented with paroxysmal generalized chorea and persistent dystonia and ataxia secondary to insulinoma-induced hypoglycemia. Her gait was wide based, and she had persistent dystonic posturing of the upper limbs (right > left; Video 1). 1 Video Segment 1: a 48-year-old woman presented with frequent episodes of paroxysmal generalized chorea involving all 4 limbs and the trunk and facio-lingual region precipitated by hypoglycemia. [Extracted from the article]

Keywords: Dystonia deafness syndrome; CAPOS syndrome; ATP1A3 mutation EN Dystonia deafness syndrome CAPOS syndrome ATP1A3 mutation 192 194 3 11/23/20 20200901 NES 200901 CAPOS syndrome (Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is one of the three overlapping clinical conditions caused by heterozygous mutations in the ATP1A3 gene, while other two include "Alternating Hemiplegia of Childhood" (AHC) and "Rapid-onset Dystonia Parkinsonism" (RDP) [1]. Dystonia deafness syndrome, CAPOS syndrome, ATP1A3 mutation. [Extracted from the article]

Background: Hemifacial spasm (HFS) is a hyperkinetic movement disorder of adults. The condition is usually primary, but may be secondary to facial nerve damage, and responds well to botulinum toxin therapy.Aim: To study the demographic and clinical features of primary and secondary HFS and assess its response to botulinum toxin therapy.Materials and Methods: A retrospective study of 40 patients of primary (33) and secondary (7) HFS, who attended our botulinum toxin clinic over the last 18 months, was conducted. We collected the data including the demographic profile, clinical features of primary and secondary HFS, severity measured by Likert scale, etiology, details of magnetic resonance imaging (MRI) of the brain, and a detailed account of the botulinum toxin injection.Results: Thirty-three out of the 40 patients had primary HFS. The mean age at presentation was 48.4 ± 12.6 years whereas the mean duration of symptoms was 57.4 ± 39.3 (6-144) months. The onset of spasm on the upper part of face was seen in 31 patients (77.5%). An ipsilateral ear click was found in 17 (42.5%) patients. Mild spasms were seen in 18 patients, whereas 12 patients had severe spasms. The severity of spasm was lower in patients with secondary HFS. Five out of 22 magnetic resonance imaging (MRI) results showed an abnormality. The mean dosage of botulinum toxin was 21.86 ± 4.19 units in primary spasm and 17 ± 4.16 units in secondary spasm (P =0.018). The mean duration of response was 112.6 ± 55.7 days (median: 90 days; range: 30-240 days) in primary spasm and 135 ± 63.6 days (median: 135 days; range: 90-180 days) in secondary spasm (P = 0.29).Conclusion: Ipsilateral auditory clicks are fairly common in primary and secondary HFS. The patients with secondary HFS have a milder intensity of disease and they may have the onset of spasm from the upper part of face. Both primary as well as secondary HFS patients respond well to botulinum toxin injection; however, the adverse effects are more likely to occur in secondary HFS, and therefore, these patients require lower dosages of toxin. [ABSTRACT FROM AUTHOR]

Additionally, tremor with a dystonia gene (ie, endophenotype of dystonia gene) may present with essential tremor-like tremor.5 Tremor has been mainly reported in dystonia associated with I DYT-ANO3 i , I DYT-KCTD17 i , I DYT-THAP1 i , I DYT-GNAL i , I DYT-TOR1A i , I DYT-KMT2B i , and I DYT-SGCE i genes.5 The dystonia in these patients can be focal or segmental without generalization and can involve laryngeal, brachial, or cranio-cervical regions with tremor and/or myoclonic jerks. Keywords: cervical dystonia; tremor EN cervical dystonia tremor 717 718 2 07/14/22 20220701 NES 220701 It is with great interest that we read the article by Kilic-Berkmen et al.1 that delineated clinical features used by movement disorder specialists in the diagnosis and classification of isolated focal cervical dystonia (CD). [Extracted from the article]

Segment 2: The patient has developed generalized hyperpigmentation of skin (more marked over face, forehead, and distal extremities) following the oral intake of tablet haloperidol. We implicated haloperidol in the causation of this hyperpigmentation as this was the only drug the patient was taking during that time. Keywords: haloperidol; neuroacanthocytosis; hyperpigmentation EN haloperidol neuroacanthocytosis hyperpigmentation 556 557 2 05/13/22 20220501 NES 220501 A 24-year-old male presented with insidious onset progressive abnormal involuntary movements of all four limbs for the last two years. [Extracted from the article]

Background Psychogenic movement disorders (PMDs) represent 2% to 20% of patients with movement disorders. There is a paucity of literature on PMD in children, with most existing literature relating to adults only. Methods For this detailed report of 58 patients (33 adults and 25 children) with PMD, history was assessed in the form of disease onset, duration, precipitating factors, diagnosis, and outcome. Movement phenotype was classified in terms of its appearance as tremor, dystonia, myoclonus, chorea, and others. Clinical evaluations were done to document variability, distractibility, and entrainment. Surface electromyography studies were obtained in patients with tremor. Results Seven adults and 7 children were classified with 'documented' PMD, and 26 adults and 18 children were classified with 'clinically established' PMD. Abrupt onset was common in adults (75.75%) and children (68%). The most common precipitating factors were family and social issues (42.42%) in adults and fear of examination (40%) and school-related issues (24%) in children. Tremor was the most common PMD in both adults and children. The second most common PMD was hemifacial spasm in adults. Conclusion The majority of patients had abrupt onset of symptoms, and tremor was the most common form of movement disorder. The second most common PMDs were hemifacial spasm in adults and dystonia and gait abnormality in children. The most common precipitating factors in adults were related to family and social issues; whereas, in children, examination and school-related issues were most common. Complete improvement was seen in only 405 of patients. [ABSTRACT FROM AUTHOR]

Levodopa‑induced dyskinesia (LID) is commonly seen in Parkinson’s disease patients treated with levodopa. This side effect is usually encountered after long duration of treatment, but occasionally, this may be seen even after few days or months of treatment. LID is broadly classified as peak‑dose dyskinesia, wearing‑off or off‑period dyskinesia, and diphasic dyskinesia. Pathogenesis of LID is complex, and different neurotransmitters such as dopamine, glutamine, adenosine, and gamma‑aminobutyric acid play important role altering the normal physiology of direct and indirect pathway of cortico‑basal ganglia‑thalamic loop responsible for fine motor control. Treatment of LID requires careful history taking and clinical examination to find the type of dyskinesia as different approach is required for different types. Changes in dopaminergic medication including continuous dopaminergic stimulation are very helpful in the management of peak‑dose dyskinesia. Different types of surgical approaches including unilateral pallidotomy and deep brain stimulation have given very good result in patients, who cannot be managed by medications alone. The surgical management of LID is dealt with in detail in another review in this series. [ABSTRACT FROM AUTHOR]

The article reports on the diagnostic approach for restless legs syndrome (RLS), also known as Willis Ekbom’s syndrome, RLS is a neurological disorder characterized by sensorimotor abnormality. It causes an abnormal unpleasant sensations involving the limbs, leading to a strong urge to move the limbs.

Commentary: Insulinoma-Induced Hypoglycemia with Generalized Chorea, Dystonia, and Ataxia: A Neurological Kaleidoscope Keywords: insulinoma; paroxysmal; hypoglycemia; dystonia; ataxia; treatable EN insulinoma paroxysmal hypoglycemia dystonia ataxia treatable S9 S10 2 09/06/21 20210802 NES 210802 Paroxysmal movement disorders (PMDs) often pose diagnostic difficulties. References 1 Mishra A, Bhattad S, Pandey S. Insulinoma-induced hypoglycemia with generalized chorea, dystonia, and ataxia: a neurological kaleidoscope. [Extracted from the article]

Keywords: spinocerebellar ataxia type 12; chorea; tremo EN spinocerebellar ataxia type 12 chorea tremo S51 S52 2 09/06/21 20210802 NES 210802 Autosomal dominant cerebellar ataxias are classified according to genetic subtype and collectively known as SCAs. GLO:JZVX/02aug21:mdc313288-fig-0001.jpg PHOTO (COLOR): 1 (i) Pedigree of patient's family shows that our patient (A), his eldest sister (B), his mother (C) and, his maternal aunt (D), are all affected and had a history of tremors of upper limbs and head tremors. Discussion Our patient had monochorea in the left upper limb which has been rarely reported in SCA12 patients. [Extracted from the article]

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Keywords: Functional; gender; movement disorders EN Functional gender movement disorders 478 479 2 05/06/20 20200501 NES 200501 We read with great interest the study by Baizabal-Carvallo and Jankovic[1] addressing the sex differences in functional movement disorders (FMDs). Baizabal-Carvallo and Jankovic[1] noticed that men and women were equally represented in patients aged 50 years or older, and thus sex differences gradually diminish after menopause in women, suggesting that the observed sex-specific disease pattern may be partly attributed to the effect of sex hormones. Although the physiological responses did not differ across sexes, the psychological measures and neuroimaging data clearly showed asymmetric prefrontal activity in men and primarily limbic activation in women.[5] The reporting bias may partly be attributed to the fact that women are more likely to report emotional stress than men. [Extracted from the article]