How to face the hemifacial spasm: challenges and misconceptions.

Hemifacial spasm (HFS) is characterised by intermittent, brief or sustained, repetitive contractions of the muscles innervated by one facial nerve. It is one of the most frequent movement disorders affecting the face. However common and allegedly straightforward to diagnose, it might reveal as a challenge for clinicians in various situations. Indeed, it often needs prior exclusion of many other movement disorders affecting the face, with frequent phenomenological overlaps with blepharospasm, post-facial palsy, facial motor tics, etc. The clinical diagnosis shall be supported by modern brain imaging techniques, and sometimes electromyography, as some particular aetiologies may require specific treatment. Primary forms are associated with vascular compression of the ipsilateral seventh cranial nerve, whereas secondary forms can be caused by any injury occurring on the facial nerve course. This article proposes a global and organised approach to the diagnosis, and the ensuing therapeutic options, as many practitioners still use some inefficient medications when they encounter a case of facial spasm.

SEVERE NEAR REFLEX SPASM IN A HEALTHY TEENAGER. A CASE REPORT.

INTRODUCTION: Spasm of the near reflex usually includes accommodative spasm, esophoria/tropia, and different degrees of miosis. Patients usually refer to distance blurred and fluctuating vision, ocular discomfort, and headaches. The diagnosis is established with refraction with and without cycloplegia; most of the cases have a functional etiology. However, some cases require neurological conditions to be ruled out; cycloplegics have an important diagnostic and therapeutic role. PURPOSE: To describe a case of bilateral severe accommodative spasm in a healthy 14-year-old teenager. CASE PRESENTATION: A 14-year-old boy with progressive diminished visual acuity attended for YSP consultation. The diagnosis of bilateral spasm of the near reflex was made, based on a gap refraction of 9.75 D between retinoscopy with and without cycloplegia and esophoria with normal keratometry and axial length. The spasm was eliminated with 2 drops of cycloplegic in each eye separated by 15 days; no clear etiology was found other than the start of school. CONCLUSION: Clinicians should be aware of pseudomyopia, especially in children with acute changes in visual acuity, who are usually exposed to myopigenic environmental factors that induce overstimulation of the parasympathetic third cranial nerve's innervation.

Is Satoyoshi syndrome an autoimmune disease? A systematic review.

OBJECTIVES: Satoyoshi syndrome is a rare multisystem disease of presumed autoimmune aetiology. We carried out a systematic review to evaluate the available evidence to support that autoimmune hypothesis. METHODS: We searched for Satoyoshi syndrome cases in PubMed, the Web of Science and Scopus up to January 2022, using keywords 'Satoyoshi syndrome' or 'Komuragaeri disease'. Data on symptoms, associated autoimmune diseases, presence of autoantibodies and response to treatment were collected. RESULTS: A total of 77 patients from 57 articles published between 1967 and 2021 were included; 59 patients were women. The mean age at diagnosis was 21.2 years. All cases had painful muscular spasms and alopecia. Frequent manifestations included: diarrhoea, malabsorption, growth retardation, amenorrhoea and bone deformity. Satoyoshi syndrome was associated with other autoimmune diseases: myasthenia gravis, autoimmune thyroiditis, idiopathic thrombocytopenic purpura, atopic dermatitis, bronchial and lupus erythematosus. Autoantibody determinations were performed in 39 patients, of which 27 had positive results. The most frequently detected autoantibodies were ANAs. Other less frequently found autoantibodies were: anti-acetylcholine receptor antibodies, anti-DNA antibodies, antithyroid antibodies, anti-glutamic acid decarboxylase (anti-GAD) and anti-gliadin antibodies. Pharmacological treatment was reported in 50 patients. Most of them improved with CS, immunosuppressants and immunoglobulins, or a combination of these medications. CONCLUSION: Satoyoshi syndrome is associated with other autoimmune diseases and a variety of autoantibodies. Improvement after CS or other immunosuppressant treatment was observed in 90% of cases. These data support an autoimmune aetiology for Satoyoshi syndrome. More studies including systematic determination of autoantibodies in all patients with Satoyoshi syndrome will help us advance in our understanding of this disease.

Effect of randomized prophylactic nitroglycerin on radial artery spasm during transradial catheterization: An analysis based on operator experience.

One limitation to transradial access (TRA) is the occurrence of spasms (RAS), for which the use of prophylactic medications is recommended. Improvement in TRA material combined with the increase in operators' expertise, might mitigate this benefit. We assess the effect of preventive nitroglycerin on RAS during TRA, evaluating the role of the operator's experience. Patients received 500 µg nitroglycerin or placebo. The operator's expertise was classified as: inexperienced (I), intermediate (M), and experienced (E). 2040 patients were included. Prophylactic use of nitroglycerin did not reduce RAS (10.8% vs. 13.4% (placebo), p = 0.07). RAS incidence was 14.5% in I, 12.5% in M, and 9.7% in E (p = 0.01). In group I, nitroglycerin reduced RAS (17.4% vs. 11.1%, p = 0.04), which was not observed in other groups. Overall, nitroglycerin does not prevent RAS, which is more common among inexperienced operators. More experienced operators could abolish preventive nitroglycerin use.

Clinical predictors and management for radial artery spasm: an Australian cross-sectional study.

INTRODUCTION: The transradial approach for coronary artery catheterisation has increased in popularity compared to the transfemoral approach for patients undergoing percutaneous coronary interventions. However, radial artery spasm continues to be a major complication of the procedure. Current management strategies vary concerning radial artery spasm and there is limited evidence of practice in the Australian context. AIM: To identify the predictors of radial artery spasm and the medications used for its prevention and management. METHODS: A descriptive cross-sectional study was carried out over a three-month period in two tertiary hospitals in NSW, Australia. A self-administered pre-procedural survey was completed by patients undergoing coronary artery catheterisation. This survey collected socio-demographic data and assessed anxiety using the Spielberger State-Trait Anxiety Inventory. Procedural data, including length of procedure, equipment used, occurrence of radial artery spasm, and medications given, were collected post-procedure by the interventionalist. RESULTS: Of the 169 participants, over half were male (59.8%) and aged 66 years or older (56.8%). Radial artery spasm was reported in 24 (14.2%) participants. Rates of spasm were significantly higher among females (66.6%, p = 0.004), those aged under 65 years (62.5%, p = 0.001) and those who reported a medical history of anxiety (33.3%, p = 0.0004). There were no significant differences in State and Trait anxiety scores among those who had RAS and those who did not. Logistic regression identified younger age as the only statistically significant predictor of RAS (OR 0.536; 95% CI 0.171-1.684; p = 0.005). To prevent radial artery spasm most patients received midazolam (n = 158; 93.5%), nitrates (n = 133; 78.7%) and/or fentanyl (n = 124; 73.4%) prophylactically. Nitrates were the most frequently administered medication to treat radial artery spasm (78.7%). CONCLUSION: This study highlights that there is a need to develop a clearer understanding of the predictors of RAS, as identifying patients at risk can ensure prophylactic measures are implemented. This study identified nitrates as the preferred vasodilator as a preventative measure along with the use of sedation.

Microvascular Decompression for Concurrent Trigeminal Neuralgia and Hemi-Laryngopharyngeal Spasm: A Case Report.

BACKGROUND AND IMPORTANCE: Hemi-laryngopharyngeal spasm (HeLPS) may result from vascular compression of the vagus nerve and can be treated using microvascular decompression. We describe the clinical characteristics and surgical treatment in a case of concurrent trigeminal neuralgia (TN) and HeLPS. CLINICAL PRESENTATION: A 44-year-old man presented with a 12-month history of right TN and 8-month history of intermittent episodes of repeated throat contractions associated with a very distressing choking sensation. Preoperative 3-dimensional fusion imaging demonstrated compression of the trigeminal nerve by the superior cerebellar artery and petrosal vein and compression of the vagus nerve by the posterior inferior cerebellar artery and one of its branches. Microvascular decompression was performed by a right standard retrosigmoid approach with enlarged craniotomy. Initially, the compression of the trigeminal nerve was identified by visual inspection. All the offending vessels were wrapped in Teflon felt, transposed away from the trigeminal nerve, and adhered to the tentorial membrane and petrous bone using fibrin glue. Furthermore, observation of the caudal rootlets of the vagus nerve revealed that the posterior inferior cerebellar artery and its branch compressed the ventral side of the nerve. These vessels were displaced antero-caudally with Teflon felt and fibrin glue; no other vessels were found around the trigeminal and vagus nerves. Postoperatively, the patient had immediate and complete resolution of symptoms of TN and HeLPS without recurrence at the 18-month follow-up. CONCLUSION: HeLPS may occur with TN, and neurosurgeons should identify the symptoms suspicious of this disease preoperatively.

Accommodative spasm and its different treatment approaches: A systematic review.

This article is about the accommodation spasm. The primary rule for near vision is ciliary muscle constriction, synchronised convergence of both eyes, and pupil constriction. Any weaknesses in these components could result in an accommodative spasm. Variable retinoscopic reflex, unstable refractive error, and lead of accommodation in near retinoscopy are common causes of spasm. We conducted a thorough literature search in the PubMed and Google Scholar databases for published journals prior to June 2022, with no data limitations. This review contains twenty-eight case reports, six cohort studies, four book references, four review articles, and two comparative studies after applying the inclusion and exclusion criteria. The majority of studies looked at accommodative spasm, near reflex spasm, and pseudomyopia. The most common causes of accommodative spasm are excessive close work, emotional distress, head injury, and strabismus. Despite side effects or an insufficient regimen, cycloplegic drops are effective in diagnosing accommodation spasm. The modified optical fogging technique is also effective and may be an option for treating accommodative spasm symptoms. Bifocals for near work, manifest refraction, base-in prisms, and vision therapy are some of the other management options. As a result, it requires a comprehensive clinical treatment strategy. This review aims to investigate the various aetiology and treatments responsible for accommodative spasm and proposes widely implementing the modified optical fogging method and vision therapy in clinics as comprehensive management to reduce the future upward trend of accommodative spasm.

[Isolated ACTH deficiency clinically presented as stiff-person syndrome, successfully treated by hormonal replacement: a case report].

A 65-year-old man was admitted to our hospital with a 6-year history of painful muscle stiffness in his trunk and lower limbs, preventing him from walking. Stiff-person syndrome (SPS) was diagnosed because the patient had symptoms of painful muscle spasms elicited by tactile stimulation without joint contracture. Although SPS- related autoantibodies in the serum, including anti-glycine R, anti-amphiphysin, anti-glutamic acid decarboxylase (GAD), anti-dipeptidyl peptidase-like protein (DPPX) and anti-γ-aminobutyric acid-A (GABAA) R, were negative, the ACTH and cortisol levels were low. On the basis of additional loading tests for anterior pituitary function and ACTH, isolated ACTH deficiency (IAD) was diagnosed. Hormonal replacement therapy with hydrocortisone at 15 mg/day ameliorated the condition quickly, and the patient became asymptomatic after three months. Flexion contractures have been reported as musculoskeletal symptoms of IAD, but are not usually evident in patients with SPS. The present case illustrates that the painful muscle spasms elicited by tactile stimulation without joint contracture characteristic of SPS can also be symptoms of IAD.

Mother-in-Child Assisted Tracking (MiCAT): A Mechanical Technique to Overcome Severe Radial Artery Spasm.

BACKGROUND: Severe radial artery spasm (RAS) and tortuosity may hinder guide catheter advancement and manipulation, requiring the arterial access site to be changed. We describe the safety and the effectiveness of a technique designed to facilitate guide catheter advancement when faced with severe RAS. METHODS AND RESULTS: We present our single-center experience using a novel mother-in-child assisted tracking (MiCAT) technique used to overcome severe RAS and facilitate guide catheter advancement to the aortic root. The MiCAT system consists of a 125 cm 4-Fr multipurpose (MP) diagnostic catheter inserted inside and protruding beyond a guiding catheter. The entire system can be advanced as one over a .035˝ guidewire into the aortic root. Procedural success was defined as achievement of the intended procedure without switching to an alternative access site with no vascular complications. The MiCAT system was utilized in 22 (0.59%) out of 3392 patients who underwent angiography between October 2019 and January 2021, with severe RAS hindering advancement of a 6 Fr guiding catheter despite conventional pharmacological therapy. MiCAT was successfully achieved via the left (55%) and right (45%) radial access routes using different 6-Fr guiding catheters. Procedural success was 100% and no vascular access or arterial complications were observed. CONCLUSIONS: In our early experience, the MiCAT technique was a safe and effective strategy to facilitate guide catheter advancement.

Impact of drug-eluting stent-associated coronary artery spasm on 3-year clinical outcomes: A propensity score matching analysis.

BACKGROUND: It has been reported that significant endothelial dysfunction or clinically evident vasospasm can be associated with drug-eluting stents (DESs). However, the impact of DES associated coronary artery spasm (CAS) on long-term clinical outcomes has not been fully elucidated as compared with those of patients with vasospastic angina. METHODS: A total of 2797 consecutive patients without significant coronary artery lesion (<70%), who underwent the Acetylcholine (Ach) provocation test, were enrolled between Nov 2004 and Oct 2010. DES-associated spasm was defined as significant CAS in proximal or distal to previously implanted DES site at follow-up angiography with Ach test. Patients were divided into two groups (DES-CAS; n = 108, CAS; n = 1878). For adjustment, propensity score matching (PSM) was done (C-statistics = 0.766, DES-CAS; n = 102, CAS; n = 102). SPSS 20 (Inc., Chicago, Illinois) was used to analyze this data. RESULTS: Baseline characteristics were worse in the DES-CAS group. After PSM, both baseline characteristics and the Ach test results were balanced except higher incidence of diffuse CAS and ECG change in the DES-CAS group. During Ach test, the incidence of diffuse spasm (93.1% vs. 81.3%, p = 0.012) and ST-T change (10.7% vs. 1.9%, p = 0.010) were higher in the DES-CAS group. At 3-year, before and after adjustment, the DES-CAS group showed a higher incidence of coronary revascularization (9.8% vs. 0.0%, p = 0.001), recurrent chest pain requiring follow up coronary angiography (CAG, 24.5% vs. 7.8%, p = 0.001) and major adverse cardiac events (MACEs, 9.8% vs. 0.9%, p < 0.005). CONCLUSION: In this study, DES associated CAS was associated with higher incidence of diffuse spasm, ST-T change and adverse 3-year clinical outcomes. Special caution should be exercised in this particular subset of patients.

Diagnosis and Management of Post Traumatic Recurrent Unilateral Accommodative Spasm-A Case Report.

Spasm of accommodation refers to constant contraction of the ciliary muscles of the eye, which fail to relax. Neurological issues, head injury, and psychogenic factors can lead to spasm of accommodation, which is generally bilateral. This case report describes the clinical presentation of traumatic, unilateral accommodative spasm in an army person. A 26-year-old male presented with complaints of diminution of near vision in the left eye noticed accidentally. History revealed a blunt injury in the eyebrow region of the left eye two months back. His best corrected visual acuity was 6/6, 0.8 M (N6) in the right eye and 6/9, 2.5 M (N18) in the left eye. Accommodative response was documented using an open-field autorefractometer that showed asymmetry in the accommodation response and pupillary diameter between the two eyes. One percent Atropine sulfate eye ointment - twice a day for 3 days - was prescribed. On the fourth day, the spasm was resolved in the left eye. Pre- and post-Atropine administration, lens thickness measurements were documented, which showed significant changes. Accommodative facility exercise was initiated after the pharmacological management. Unilateral accommodative spasm is rare and needs careful investigations. Objective assessment of accommodative response and lens thickness measurement play a vital role in confirming the diagnosis.

Extent of EEG monitoring for detecting epileptic spasms.

INTRODUCTION: Epileptic spasms (ES) are the ictal manifestation of West syndrome. Due to poor interrater reliability in diagnosing hypsarrhythmia, identification of ES - ideally on video EEG monitoring - is important to start proper treatment. METHODS: We retrospectively analyzed inpatient video EEG recordings of 42 patients with new onset ES and calculated the time needed to capture the first ES, along with time to first sleep epoch, among other data. RESULTS: The average time to capture ES was 188.36 min with a 95 percent confidence interval from 116.95 to 259.76 min. The average onset of sleep occurred at 30.8 min with 95% Confidence interval between 19.9 and 41.9 min. There was no significant correlation between duration of symptoms and etiology and the time needed to detect first spasm on EEG. CONCLUSIONS: Routine or short duration EEGs are not sufficient to capture ES in most patients. 4-5-hour VEEG monitoring duration should be considered to capture ictal data in patients suspected of having ES.

Palmaris brevis spasm syndrome in manual workers using vibratory tools.

Palmaris brevis spasm syndrome (PBSS) appears to reflect benign muscle hyperactivity, with unprovoked recurrent spasm of the muscle and consequent dimpling of the hypothenar eminence. Two cases are reported, being the first known report of the condition among manual workers or those using vibratory tools. Each was diagnosed during routine health surveillance for hand-arm vibration syndrome. With hand cramps being a frequent report among vibration-exposed workers, this condition should be considered in such cases, although the lack of reports means that the frequency of the condition is unknown. One of the employees described more frequent episodes of spasm during periods of increased manual work. Diagnosis is possible by witnessing the characteristic spasm, or by viewing photographs of it. Functional impairment is not usual, except for the brief duration of an attack. There is no consensus regarding appropriate treatment.

Status of epileptic spasms: A study of 21 children.

OBJECTIVE: We studied cases with long-lasting epileptic spasms (ES) considered as a spasm status analyzing type of epilepsy, epileptic syndrome, etiology, treatment, and outcome in 21 patients. METHODS: We evaluated the charts of 21 patients seen between June 2006 and July 2017 who met the electroclinical diagnostic criteria of a spasm status. The spasm status was defined as continuous ES lasting 30 min or longer. RESULTS: The type of ES was mixed in nine patients, flexion in seven, and extension in five. Epileptic spasms were asymmetric in three patients and unilateral in two. They occurred on awakening in all patients, while during sleep they decreased in all and disappeared in three cases. The duration of the spasm status ranged from 40 min to 15 days according to the seizure diaries of patients and video-EEG recordings. Two well-defined subgroups of patients were recognized; the first included patients with West syndrome (WS) and the second other types of severe non-West epilepsy syndromes. The spasm status responded well to oral vigabatrin (VGB) in four patients, oral topiramate (TPM) in three, oral corticosteroids in one, and cannabidiol in another patient. A good response was observed with benzodiazepines in six patients, with phenytoin (PH) in two, and with phenobarbital (PB) in one. Adrenocorticotropic hormone (ACTH) was effective in one patient and the ketogenic diet in two. Prognosis depends on the etiology. CONCLUSION: In this study we identified patients with WS and other types of severe non-West epilepsy syndromes who had a particular type of long-lasting ES that, in spite of its long duration does not strictly meet the criteria of the International League against Epilepsy (ILAE) classification of status epilepticus, may be considered a spasm status.

Functional convergence spasm: an unexpected finding in a patient with focal epilepsy.

OBJECTIVE: Convergence spasm is a clinical condition characterized by transient episodes of convergence, miosis and accommodation with strabismus and diplopia and it is usually a manifestation of a functional neurological disorder. We describe a patient with a challenging diagnosis of convergence spasm in the setting of occipital lobe epilepsy. CASE REPORT: A 52-year-old woman came for the assessment of focal epilepsy due to left occipital cortical dysplasia. During ocular motility tests, she presented with episodes of short duration (~10-30 seconds) of convergent strabismus. Neuropsychological evaluation showed a severe mixed anxiety-depressive disorder with a tendency toward somatization. RESULTS: Convergence spasm was recorded during video-EEG examination and no ictal activity was present. CONCLUSIONS: To our knowledge, no other report of functional convergence spasm in the context of focal epilepsy associated with cortical dysplasia has been described in literature.

Repetitive sleep starts: An important differential diagnosis of infantile spasms.

OBJECTIVE: Repetitive sleep starts (RSS) are clusters of nonepileptic, spasm-like movements occurring during sleep onset. However, their characteristics have yet to be defined. We conducted a clinicoelectroencephalographic study of children with RSS to clarify their detailed characteristics. METHODS: To differentiate starts from epileptic spasms, we recruited children with brief "crescendo-decrescendo" muscle contractions that simultaneously involved the limbs and trunk without electroencephalogram changes, and that fulfilled the following criteria: (1) repeated occurrence (five or more) and (2) manifestation during sleep stage N1-N2. A total of nine children met these criteria. Their clinical information and video-electroencephalogram data were analyzed retrospectively. RESULTS: The background conditions observed at onset of RSS were perinatal hypoxic-ischemic encephalopathy (n = 4), West syndrome of unknown etiology (n = 1), and traumatic brain injury (n = 1). The age at onset of RSS, the number of starts in a given RSS cluster, the interval between starts, and the duration of surface electromyogram activity were between 3 and 46 months, 5 and 547, <1 and 60 s, and 0.3 and 5.4 s, respectively. None of the median value of these parameters differed between children with and without corticospinal tract injury. During the median follow-up period of 33 months, RSS disappeared spontaneously in five. CONCLUSION: This is the largest case series of RSS clarifying their clinicoelectroencephalographic characteristics reported to date. To avoid unnecessary antiepileptic therapies, clinicians should be aware of RSS and distinguish it from other disorders involving involuntary movements or seizures, especially epileptic spasms.

Tetanus presenting as painful muscle spasms, dysphagia and delirium in an older adult.

While tetanus is now a rare disease in the UK, it remains an important differential diagnosis for trismus and muscle spasms. Even more so in older adults, as this population is less likely to have received full vaccination. Hence, the highest incidence of tetanus in England is seen in older adults. Written informed consent for publication of their clinical details was obtained from the patient proxy.

Isolated Pyridoxine Deficiency Presenting as Muscle Spasms in a Patient With Type 2 Diabetes: A Case Report and Literature Review.

Pyridoxine is an important co-factor for many biochemical reactions in cellular metabolism related to the synthesis and catabolism of amino acids, fatty acids, neurotransmitters. Deficiency of pyridoxine results in impaired transcellular signaling between neurons and presents with muscular convulsions, hyperirritability, and peripheral neuropathy. Deficiency of pyridoxine is usually found in association with other vitamin B deficiencies such as folate (vitamin B9) and cobalamin (vitamin B12). Isolated pyridoxine deficiency is extremely rare. We present the case of a 59-year old female with type 2 diabetes who complained of painful muscle spasms. Her muscle spasms involved in both feet, which have spread proximally to her legs. She also experienced intermittent muscle spasms in her left arm, which is not alleviated by baclofen, cyclobenzaprine. Her plasma pyridoxal 5-phosphate confirmed pyridoxine deficiency. Vitamins B1, B3, B12, and folate were within normal limits. The patient received standard-dose intramuscular pyridoxine injections for three weeks followed by oral supplements for 3 months and her symptoms resolved. This case illustrates the rare instance of isolated pyridoxine deficiency in type 2 diabetes patient manifesting as myoclonic muscle spasms involving the legs and arms in the absence of objective polyneuropathy. Pyridoxine level should, therefore, be assessed in patients with type 2 diabetes, including newly diagnosed patients.

Diagnosis of coronary artery spasm by ergonovine provocation test of radial artery.

We investigated the sensitivity, specificity and safety of ergonovine provocation test of radial artery in the diagnosis of coronary artery spasm (CAS). The patients who came to our hospital for chest pain from January to June 2020 as well as had coronary stenosis < 50% and no radial artery stenosis, were enrolled in this study. These patients were divided into CAS group and control group after intracoronary ergonovine provocation test. All patients underwent ergonovine provocation test of radial artery, the inner diameter (D0 and D1) and the peak systolic velocities (PSV0 and PSV1) of the radial artery were measured by ultrasound before and after ergonovine provocation. The predictive value of ergonovine provocation test of radial artery for the diagnosis of CAS was analyzed using receiver operator characteristic (ROC) curve. There were 19 patients in the CAS group and 28 patients in the control group. Low density lipoprotein cholesterol and smoking rate were significantly higher in the CAS group than in the control group (all P < 0.05), but there were no significant differences in other items (P > 0.05) between the two groups. In the ergonovine provocation test of radial artery, degree of radial artery stenosis was significantly higher in the CAS group [41.50% (35.60%, 50.00%)] than in the control group [11.25% (5.15%, 23.00%)] (P = 0.000), but there were no siginificant differences in D0, PSV0 and PSV1 between the two groups (P > 0.05). The area under ROC curve of ergonovine (120 µg) provocation test of radial artery for the diagnosis of CAS was 0.912 with 95%CI: 0.792-0.975, P = 0.001, cut-off of 31%, specificity of 92.86% and sensitivity of 84.21%. The ergonovine (120 µg) provocation test of radial artery did not cause any adverse reactions. We concluded that the ergonovine provocation test of radial artery has high sensitivity, specificity and safety in the diagnosis of CAS.