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2.
J Neurophysiol ; 132(3): 953-967, 2024 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-39110516

RESUMO

Deep brain stimulation (DBS) of the internal segment of the globus pallidus (GPi) can markedly reduce muscle rigidity in people with Parkinson's disease (PD); however, the mechanisms mediating this effect are poorly understood. Computational modeling of DBS provides a method to estimate the relative contributions of neural pathway activations to changes in outcomes. In this study, we generated subject-specific biophysical models of GPi DBS (derived from individual 7-T MRI), including pallidal efferent, putamenal efferent, and internal capsule pathways, to investigate how activation of neural pathways contributed to changes in forearm rigidity in PD. Ten individuals (17 arms) were tested off medication under four conditions: off stimulation, on clinically optimized stimulation, and on stimulation specifically targeting the dorsal GPi or ventral GPi. Quantitative measures of forearm rigidity, with and without a contralateral activation maneuver, were obtained with a robotic manipulandum. Clinically optimized GPi DBS settings significantly reduced forearm rigidity (P < 0.001), which aligned with GPi efferent fiber activation. The model demonstrated that GPi efferent axons could be activated at any location along the GPi dorsal-ventral axis. These results provide evidence that rigidity reduction produced by GPi DBS is mediated by preferential activation of GPi efferents to the thalamus, likely leading to a reduction in excitability of the muscle stretch reflex via overdriving pallidofugal output.NEW & NOTEWORTHY Subject-specific computational models of pallidal deep brain stimulation, in conjunction with quantitative measures of forearm rigidity, were used to examine the neural pathways mediating stimulation-induced changes in rigidity in people with Parkinson's disease. The model uniquely included internal, efferent and adjacent pathways of the basal ganglia. The results demonstrate that reductions in rigidity evoked by deep brain stimulation were principally mediated by the activation of globus pallidus internus efferent pathways.


Assuntos
Estimulação Encefálica Profunda , Globo Pálido , Rigidez Muscular , Doença de Parkinson , Humanos , Globo Pálido/fisiopatologia , Globo Pálido/fisiologia , Doença de Parkinson/terapia , Doença de Parkinson/fisiopatologia , Rigidez Muscular/fisiopatologia , Rigidez Muscular/terapia , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Vias Neurais/fisiopatologia , Vias Neurais/fisiologia , Modelos Neurológicos
3.
Continuum (Minneap Minn) ; 30(4): 1110-1135, 2024 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-39088290

RESUMO

OBJECTIVE: Antibodies against glutamic acid decarboxylase (GAD), originally associated with stiff person syndrome (SPS), define the GAD antibody-spectrum disorders that also include cerebellar ataxia, autoimmune epilepsy, limbic encephalitis, progressive encephalomyelitis with rigidity and myoclonus (PERM), and eye movement disorders, all of which are characterized by autoimmune neuronal excitability. This article elaborates on the diagnostic criteria for SPS and SPS spectrum disorders, highlights disease mimics and misdiagnoses, describes the electrophysiologic mechanisms and underlying autoimmunity of stiffness and spasms, and provides a step-by-step therapeutic scheme. LATEST DEVELOPMENTS: Very-high serum GAD antibody titers are diagnostic for GAD antibody-spectrum disorders and also predict the presence of GAD antibodies in the CSF, increased intrathecal synthesis, and reduced CSF γ-aminobutyric acid (GABA) levels. Low serum GAD antibody titers or the absence of antibodies generates diagnostic challenges that require careful distinction in patients with a variety of painful spasms and stiffness, including functional neurologic disorders. Antibodies against glycine receptors, first found in patients with PERM, are seen in 13% to 15% of patients with SPS, whereas amphiphysin and gephyrin antibodies, seen in 5% of patients with SPS spectrum disorders, predict a paraneoplastic association. GAD-IgG from different SPS spectrum disorders recognizes the same dominant GAD intracellular epitope and, although the pathogenicity is unclear, is an excellent diagnostic marker. The biological basis of muscle stiffness and spasms is related to autoimmune neuronal hyperexcitability caused by impaired reciprocal γ-aminobutyric acid-mediated (GABA-ergic) inhibition, which explains the therapeutic response to GABA-enhancing agents and immunotherapies. ESSENTIAL POINTS: It is essential to distinguish SPS spectrum disorders from disease mimics to avoid both overdiagnoses and misdiagnoses, considering that SPS is treatable if managed correctly from the outset to prevent disease progression. A step-by-step, combination therapy of GABA-enhancing medications along with immunotherapies ensures prolonged clinical benefits.


Assuntos
Autoanticorpos , Glutamato Descarboxilase , Rigidez Muscular Espasmódica , Humanos , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/imunologia , Rigidez Muscular Espasmódica/fisiopatologia , Rigidez Muscular Espasmódica/sangue , Glutamato Descarboxilase/imunologia , Autoanticorpos/sangue , Masculino , Feminino , Rigidez Muscular/diagnóstico , Rigidez Muscular/imunologia , Rigidez Muscular/tratamento farmacológico , Encefalomielite/diagnóstico , Encefalomielite/imunologia , Encefalomielite/sangue , Pessoa de Meia-Idade , Adulto , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/imunologia , Ataxia Cerebelar/sangue , Ataxia Cerebelar/fisiopatologia , Encefalite Límbica/diagnóstico , Encefalite Límbica/imunologia , Encefalite Límbica/terapia , Encefalite Límbica/sangue , Encefalite Límbica/fisiopatologia
4.
Neurol Neuroimmunol Neuroinflamm ; 11(6): e200298, 2024 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-39213470

RESUMO

OBJECTIVES: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder that can be associated with antibodies against surface antigens (glycine receptor (GlyR), dipeptidyl-peptidase-like-protein-6) and intracellular antigens (glutamate decarboxylase (GAD65), amphiphysin). METHODS: We report clinico-pathologic findings of a PERM patient with coexisting GlyR and GAD65 antibodies. RESULTS: A 75-year-old man presented with myoclonus and pain of the legs, subsequently developed severe motor symptoms, hyperekplexia, a pronounced startle reflex, hallucinations, dysautonomia, and died 10 months after onset despite extensive immunotherapy, symptomatic treatment, and continuous intensive care support. Immunotherapy comprised corticosteroids, IVIG, plasmapheresis, immunoadsorption, cyclophosphamide, and bortezomib. Intensive care treatment and permanent isoflurane sedation was required for more than 20 weeks. CNS tissue revealed neuronal loss, astrogliosis and microgliosis, representing a pallido-nigro-dentato-bulbar-spinal degeneration pattern, specifically along GlyR and GAD expression sites. Neurons showed pSTAT1, MHC class I, and GRP78 upregulation. Inflammation was moderate and characterized by CD8+ T cells and single CD20+/CD79a+ B/plasma cells. Focal tau-positive thread-like deposits were detected in gliotic brainstem areas. In the spinal cord, GlyR, glycine transporter-2, and GAD67 expression were strongly reduced. DISCUSSION: A possible potentiating effect of pathogenic GlyR antibodies together with T cells directed against neurons may have led to the severe and progressive clinical course.


Assuntos
Autoanticorpos , Encefalomielite , Glutamato Descarboxilase , Rigidez Muscular , Mioclonia , Receptores de Glicina , Humanos , Masculino , Idoso , Glutamato Descarboxilase/imunologia , Rigidez Muscular/etiologia , Rigidez Muscular/imunologia , Autoanticorpos/sangue , Encefalomielite/imunologia , Encefalomielite/complicações , Mioclonia/etiologia , Receptores de Glicina/imunologia , Rigidez Muscular Espasmódica/imunologia , Rigidez Muscular Espasmódica/complicações , Evolução Fatal
5.
BMJ Case Rep ; 17(8)2024 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-39122374

RESUMO

Stiff-person syndrome (SPS) usually manifests as an autoimmune neuromuscular disorder characterised by pronounced and advancing rigidity, primarily affecting the trunk and proximal muscles. There are various clinical subtypes like classic SPS (truncal stiffness, generalised rigidity and muscle spasms), partial SPS (stiff-limb syndrome) and uncommon forms including progressive encephalomyelitis with rigidity and myoclonus. Camptocormia, defined as forward flexion of the spine in the upright position that disappears in the supine position, without fixed deformity, has been described only in two cases as an initial presentation of Anti glutamic acid decarboxylase (GAD) autoimmunity. We encountered a young male presenting with a progressive forward-leaning posture and involuntary rhythmic movements in the lower limb. Diagnostic workup included MRI, blood routines, autoimmune screening, genetic testing, lumbar puncture and electromyography. Elevated serum anti-GAD antibody levels, inflammatory CSF and certain other clinical features supported the diagnosis of SPS. Treatment involved benzodiazepines, muscle relaxants and immunotherapy with intravenous immunoglobulin. This case underscores the importance of considering immune-mediated causes, such as SPS, in patients presenting with camptocormia.


Assuntos
Glutamato Descarboxilase , Atrofia Muscular Espinal , Curvaturas da Coluna Vertebral , Rigidez Muscular Espasmódica , Humanos , Masculino , Rigidez Muscular Espasmódica/imunologia , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/complicações , Curvaturas da Coluna Vertebral/imunologia , Curvaturas da Coluna Vertebral/complicações , Curvaturas da Coluna Vertebral/etiologia , Glutamato Descarboxilase/imunologia , Atrofia Muscular Espinal/diagnóstico , Atrofia Muscular Espinal/complicações , Atrofia Muscular Espinal/imunologia , Autoanticorpos/sangue , Autoanticorpos/imunologia , Imunoglobulinas Intravenosas/uso terapêutico , Adulto , Rigidez Muscular/imunologia , Rigidez Muscular/tratamento farmacológico , Rigidez Muscular/diagnóstico , Eletromiografia , Imageamento por Ressonância Magnética
6.
Front Immunol ; 15: 1387591, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38953026

RESUMO

Background and objectives: Antiglycine receptor (anti-GlyR) antibody mediates multiple immune-related diseases. This study aimed to summarize the clinical features to enhance our understanding of anti-GlyR antibody-related disease. Methods: By collecting clinical information from admitted patients positive for glycine receptor (GlyR) antibody, the clinical characteristics of a new patient positive for GlyR antibody were reported in this study. To obtain additional information regarding anti-GlyR antibody-linked illness, clinical data and findings on both newly reported instances in this study and previously published cases were merged and analyzed. Results: A new case of anti-GlyR antibody-related progressive encephalomyelitis with rigidity and myoclonus (PERM) was identified in this study. A 20-year-old man with only positive cerebrospinal fluid anti-GlyR antibody had a good prognosis with first-line immunotherapy. The literature review indicated that the common clinical manifestations of anti-GlyR antibody-related disease included PERM or stiff-person syndrome (SPS) (n = 179, 50.1%), epileptic seizure (n = 94, 26.3%), and other neurological disorders (n = 84, 24.5%). Other neurological issues included demyelination, inflammation, cerebellar ataxia and movement disorders, encephalitis, acute psychosis, cognitive impairment or dementia, celiac disease, Parkinson's disease, neuropathic pain and allodynia, steroid-responsive deafness, hemiballism/tics, laryngeal dystonia, and generalized weakness included respiratory muscles. The group of PERM/SPS exhibited a better response to immunotherapy than others. Conclusions: The findings suggest the presence of multiple clinical phenotypes in anti-GlyR antibody-related disease. Common clinical phenotypes include PERM, SPS, epileptic seizure, and paraneoplastic disease. Patients with RERM/SPS respond well to immunotherapy.


Assuntos
Autoanticorpos , Encefalomielite , Rigidez Muscular , Receptores de Glicina , Humanos , Masculino , Receptores de Glicina/imunologia , Autoanticorpos/imunologia , Autoanticorpos/sangue , Adulto Jovem , Encefalomielite/imunologia , Encefalomielite/diagnóstico , Rigidez Muscular/imunologia , Rigidez Muscular/etiologia , Rigidez Muscular/diagnóstico , Mioclonia/imunologia , Mioclonia/diagnóstico , Rigidez Muscular Espasmódica/imunologia , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/terapia , Adulto
7.
BMC Pediatr ; 24(1): 427, 2024 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-38961420

RESUMO

BACKGROUND: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and life-threatening autoimmune disease of the central nervous system. So far, only ten cases of PERM have been reported in children worldwide, including the one in this study. CASE PRESENTATION: We report a case of an 11-year-old boy with PERM with an initial presentation of abdominal pain, skin itching, dysuria, urinary retention, truncal and limb rigidity, spasms of the trunk and limbs during sleep, deep and peripheral sensory disturbances, and dysphagia. A tissue-based assay using peripheral blood was positive, demonstrated by fluorescent staining of mouse cerebellar sections. He showed gradual and persistent clinical improvement after immunotherapy with intravenous immunoglobulin, steroids, plasmapheresis and rituximab. CONCLUSIONS: We summarized the diagnosis and treatment of a patient with PERM and performed a literature review of pediatric PERM to raise awareness among pediatric neurologists. A better comprehension of this disease is required to improve its early diagnosis, treatment, and prognosis.


Assuntos
Encefalomielite , Rigidez Muscular , Mioclonia , Humanos , Masculino , Criança , Rigidez Muscular/etiologia , Encefalomielite/diagnóstico , Encefalomielite/complicações , Mioclonia/etiologia , Mioclonia/diagnóstico
8.
J Neurol ; 271(8): 5223-5232, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38839639

RESUMO

Video head impulse tests (video-HITs) are commonly used for vestibular evaluation; however, the results can be contaminated by various artifacts, including technical errors, recording problems, and participant factors. Although video-HITs can be used in patients with Parkinson's disease (PD), the effect of neck rigidity has not been systematically investigated. This study aimed to investigate the effect of neck rigidity on video-HIT results in patients with PD. We prospectively recruited 140 consecutive patients with PD (mean age ± standard deviation = 68 ± 10 years, 69 men) between September 2021 and April 2024 at Korea University Medical Center. The video-HIT results were compared with those of 19 age- and sex-matched healthy participants. Neck rigidity was stratified as a subdomain of the Movement Disorder Society-Unified Parkinson's Disease Rating Scale motor part (MDS-UPDRS-III). In 59 patients, the vestibulo-ocular reflex (VOR) gain was overestimated in at least one canal plane (58/140, 41%), mostly in the anterior canal (AC, n = 44), followed by the horizontal (HC, n = 15) and posterior canals (PC, n = 7). VOR gain overestimation was also observed in patients with no (18/58, 35%), subtle (20/58, 34%), or mild (17/58, 29%) neck rigidity. Multivariable logistic regression analysis showed that VOR overestimation was positively associated with neck rigidity (odds ratio [OR] [95% confidence interval] = 1.51 [1.01-2.25], p = 0.043). The head velocities of patients decreased during head impulses for the AC (p = 0.033 for the right AC; p = 0.014 for the left AC), whereas eye velocities were similar to those of healthy participants. Our findings suggest that neck rigidity may be a confounder that can contaminate video-HIT results. Thus, the results of video-HITs, especially for the AC, should be interpreted with the context of head velocity during head impulses in patients with neck rigidity.


Assuntos
Teste do Impulso da Cabeça , Rigidez Muscular , Doença de Parkinson , Reflexo Vestíbulo-Ocular , Gravação em Vídeo , Humanos , Doença de Parkinson/fisiopatologia , Doença de Parkinson/diagnóstico , Masculino , Feminino , Idoso , Teste do Impulso da Cabeça/métodos , Pessoa de Meia-Idade , Reflexo Vestíbulo-Ocular/fisiologia , Rigidez Muscular/etiologia , Rigidez Muscular/fisiopatologia , Rigidez Muscular/diagnóstico , Estudos Prospectivos , Músculos do Pescoço/fisiopatologia
9.
Ann Neurol ; 96(2): 234-246, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38721781

RESUMO

OBJECTIVE: Bradykinesia and rigidity are considered closely related motor signs in Parkinson disease (PD), but recent neurophysiological findings suggest distinct pathophysiological mechanisms. This study aims to examine and compare longitudinal changes in bradykinesia and rigidity in PD patients treated with bilateral subthalamic nucleus deep brain stimulation (STN-DBS). METHODS: In this retrospective cohort study, the clinical progression of appendicular and axial bradykinesia and rigidity was assessed up to 15 years after STN-DBS in the best treatment conditions (ON medication and ON stimulation). The severity of bradykinesia and rigidity was examined using ad hoc composite scores from specific subitems of the Unified Parkinson's Disease Rating Scale motor part (UPDRS-III). Short- and long-term predictors of bradykinesia and rigidity were analyzed through linear regression analysis, considering various preoperative demographic and clinical data, including disease duration and severity, phenotype, motor and cognitive scores (eg, frontal score), and medication. RESULTS: A total of 301 patients were examined before and 1 year after surgery. Among them, 101 and 56 individuals were also evaluated at 10-year and 15-year follow-ups, respectively. Bradykinesia significantly worsened after surgery, especially in appendicular segments (p < 0.001). Conversely, rigidity showed sustained benefit, with unchanged clinical scores compared to preoperative assessment (p > 0.05). Preoperative motor disability (eg, composite scores from the UPDRS-III) predicted short- and long-term outcomes for both bradykinesia and rigidity (p < 0.01). Executive dysfunction was specifically linked to bradykinesia but not to rigidity (p < 0.05). INTERPRETATION: Bradykinesia and rigidity show long-term divergent progression in PD following STN-DBS and are associated with independent clinical factors, supporting the hypothesis of partially distinct pathophysiology. ANN NEUROL 2024;96:234-246.


Assuntos
Estimulação Encefálica Profunda , Hipocinesia , Rigidez Muscular , Doença de Parkinson , Núcleo Subtalâmico , Humanos , Doença de Parkinson/terapia , Doença de Parkinson/fisiopatologia , Doença de Parkinson/complicações , Estimulação Encefálica Profunda/efeitos adversos , Estimulação Encefálica Profunda/métodos , Masculino , Feminino , Hipocinesia/etiologia , Hipocinesia/fisiopatologia , Pessoa de Meia-Idade , Núcleo Subtalâmico/fisiopatologia , Rigidez Muscular/etiologia , Rigidez Muscular/fisiopatologia , Idoso , Estudos Retrospectivos , Progressão da Doença , Estudos de Coortes
10.
Nat Commun ; 15(1): 4662, 2024 May 31.
Artigo em Inglês | MEDLINE | ID: mdl-38821913

RESUMO

Deep Brain Stimulation can improve tremor, bradykinesia, rigidity, and axial symptoms in patients with Parkinson's disease. Potentially, improving each symptom may require stimulation of different white matter tracts. Here, we study a large cohort of patients (N = 237 from five centers) to identify tracts associated with improvements in each of the four symptom domains. Tremor improvements were associated with stimulation of tracts connected to primary motor cortex and cerebellum. In contrast, axial symptoms are associated with stimulation of tracts connected to the supplementary motor cortex and brainstem. Bradykinesia and rigidity improvements are associated with the stimulation of tracts connected to the supplementary motor and premotor cortices, respectively. We introduce an algorithm that uses these symptom-response tracts to suggest optimal stimulation parameters for DBS based on individual patient's symptom profiles. Application of the algorithm illustrates that our symptom-tract library may bear potential in personalizing stimulation treatment based on the symptoms that are most burdensome in an individual patient.


Assuntos
Estimulação Encefálica Profunda , Córtex Motor , Doença de Parkinson , Tremor , Humanos , Estimulação Encefálica Profunda/métodos , Doença de Parkinson/terapia , Doença de Parkinson/fisiopatologia , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Tremor/terapia , Tremor/fisiopatologia , Córtex Motor/fisiopatologia , Algoritmos , Hipocinesia/terapia , Hipocinesia/fisiopatologia , Substância Branca/patologia , Substância Branca/fisiopatologia , Rigidez Muscular/terapia , Cerebelo/fisiopatologia , Estudos de Coortes , Resultado do Tratamento
11.
Clin Toxicol (Phila) ; 62(4): 267-268, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38738682

RESUMO

INTRODUCTION: There has been a previous case report of peri-arrest muscle rigidity in the setting of severe salicylate poisoning (serum salicylate concentration 1,500 mg/L), described as paratonia or rapid rigor mortis. We present an image of rapid rigor mortis in another fatal salicylate poisoning. CASE SUMMARY: We report a 42-year-old male with severe salicylate poisoning (peak salicylate concentration 1,600 mg/L). During the peri-arrest period, the patient developed isotonic flexion of the upper and lower extremities, the clinical signs of rapid-occurring rigor mortis. Despite resuscitative efforts, the patient died. IMAGE: Our patient is exhibiting peri-arrest rigidity in the upper extremities. DISCUSSION: Peri-mortem rigidity is due to depletion of adenosine triphosphate. Severe salicylate poisoning causes uncoupling of oxidative phosphorylation which prevents the production of adenosine triphosphate, which is required to release myosin from actin to allow the muscle to relax. A limitation of our report is that we did not definitively exclude other uncouplers of oxidative phosphorylation, such as 2,4-dinitrophenol. However, the history of aspirin ingestion was provided by the patient and corroborated by his mother, and it was confirmed by measurement of his salicylate concentration. CONCLUSION: We hypothesize that in our patient, rapid-occurring rigor mortis likely resulted from depletion of adenosine triphosphate. This occurred as a result of uncoupling of oxidative phosphorylation in the mitochondria from severe salicylate poisoning, as adenosine triphosphate is required for muscle relaxation.


Assuntos
Rigidez Muscular , Salicilatos , Humanos , Masculino , Adulto , Rigidez Muscular/induzido quimicamente , Salicilatos/intoxicação , Salicilatos/sangue , Evolução Fatal , Autopsia , Aspirina/intoxicação
12.
MedEdPORTAL ; 20: 11389, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38560702

RESUMO

Introduction: Tetanus is uncommon in the United States secondary to vaccination. However, vaccination hesitancy is increasing. This case challenges medical students to consider tetanus in the differential and understand its complications. Methods: Fourth-year medical students took a pretest on the neurotransmitter glycine and associated disease states. They received two 10-minute lectures on glycine and acid-base abnormalities. Students then participated in a simulation featuring a 27-year-old man bitten by a dog, resulting in tetanus. Required equipment included a mannequin with monitor, a defibrillator, and personal protective equipment. Critical actions consisted of learners dividing up roles amongst each other, using closed-loop communication, placing the patient on a cardiac monitor, choosing to establish IV access and intubate the patient, starting IV fluids, and administering tetanus immunoglobulin. The case ended after 20 minutes. Outcome measurements encompassed performance on a posttest and critical actions. Results: Twenty students participated. Mean pretest and posttest scores were 69.5 and 92.5, respectively (p < .001). All groups completed the items on the critical actions checklist within a 20-minute time frame. Discussion: Rising vaccine hesitancy may increase the likelihood of physicians encountering new cases of tetanus and require them to perform lifesaving management of a patient presenting with muscle rigidity. This simulation provides learners with hands-on experience caring for a patient with tetanus and muscle rigidity. It can improve their knowledge of recognition, assessment, and decision-making toward lifesaving management of tetanus by allowing them to practice their skills in a safe environment.


Assuntos
Estudantes de Medicina , Tétano , Masculino , Humanos , Estados Unidos , Animais , Cães , Adulto , Tétano/complicações , Tétano/diagnóstico , Rigidez Muscular , Simulação por Computador , Glicina
13.
Pain Pract ; 24(5): 798-804, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38185725

RESUMO

BACKGROUND: Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by rigidity and painful spasm primarily affecting the truncal and paraspinal musculature due to autoimmune-mediated neuronal hyperexcitability. Spinal cord stimulation (SCS) is an approved therapy for managing painful neuropathic conditions, including diabetic peripheral neuropathy and refractory angina pectoris. We describe the novel use of SCS for the treatment of spasm and rigidity in a 49-year-old man with seropositive stiff person syndrome (SPS). The patient was treated with intravenous immunoglobulin (IVIG) and oral medications over a 13-month period with minimal improvement, prompting consideration of SCS. To our knowledge, this is the first report of the successful use of SCS in SPS with the demonstration of multifaceted clinical improvement. METHODS: Following a successful temporary SCS trial, permanent implantation was performed. Spasm/stiffness (Distribution of Stiffness Index; Heightened Sensitivity Scale; Penn Spasm Frequency Scale, PSFS), disability (Oswestry Disability Index, ODI; Pain Disability Index, PDI), depression (Patient Health Questionnaire-9, PHQ-9), sleep (Pittsburgh Sleep Quality Index, PSQI), fatigue (Fatigue Severity Scale, FSS), pain (Numerical Pain Rating Scale, NPRS), quality of life (EuroQoL 5 Dimension 5 Level, EQ-5D-5L), and medication usage were assessed at baseline, 6-month, and 10-month postimplantation. RESULTS: ODI, PHQ-9, FSS, NPRS, PSQI, and EQ-5D-5L scores showed a notable change from baseline and surpassed the defined minimal clinically important difference (MCID) at 6-month and 10-month follow-up. Oral medication dosages were reduced. CONCLUSIONS: The novel use of SCS therapy in seropositive SPS resulted in functional improvement and attenuation of symptoms. We present possible mechanisms by which SCS may produce clinical response in patients with SPS and aim to demonstrate proof-of-concept for a future comprehensive pilot study evaluating SCS-mediated response in SPS.


Assuntos
Estimulação da Medula Espinal , Rigidez Muscular Espasmódica , Humanos , Rigidez Muscular Espasmódica/terapia , Rigidez Muscular Espasmódica/complicações , Masculino , Pessoa de Meia-Idade , Estimulação da Medula Espinal/métodos , Rigidez Muscular/terapia , Rigidez Muscular/etiologia , Espasmo/terapia , Espasmo/etiologia , Resultado do Tratamento
15.
Vet Med Sci ; 9(6): 2404-2409, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37846941

RESUMO

A young stray entire female domestic shorthair cat was presented with symmetrical forelimb extensor rigidity, neck hyperextension and hindlimb paraplegia, characteristic of Schiff-Sherrington phenomenon (SSP), within 30 min of a motor vehicle accident. Radiographic and post-mortem studies disclosed complete transection of the spinal cord from traumatic dorsocranial luxation of the second lumbar vertebra, displacement of the sacrum from the ilium, seventh lumbar and first caudal vertebrae and multiple pelvic fractures. Other causes of forelimb extensor rigidity and neck hyperextension such as decerebrate and decerebellate rigidity were excluded by a lack of neurological signs consistent with these entities and unremarkable findings on post-mortem examination of the cranial cavity and brain and histological examination of the cerebrum, brainstem and cerebellum. To the best of the author's knowledge, this is the first report of SSP in the cat outside the experimental arena of decerebrate or non-decerebrate preparations following post-brachial spinal cord transection/cold block.


Assuntos
Doenças do Gato , Traumatismos da Medula Espinal , Feminino , Gatos , Animais , Rigidez Muscular/veterinária , Traumatismos da Medula Espinal/diagnóstico por imagem , Traumatismos da Medula Espinal/etiologia , Traumatismos da Medula Espinal/cirurgia , Traumatismos da Medula Espinal/veterinária , Coluna Vertebral , Doenças do Gato/diagnóstico por imagem , Doenças do Gato/cirurgia
16.
J Emerg Med ; 65(4): e307-e309, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37690955

RESUMO

BACKGROUND: Acute calcific tendinitis (ACT) of the longus colli muscle (LCM) is an inflammatory response due to deposition of calcium hydroxyapatite crystals. It is typically correlated with whiplash and overuse injuries. A common presentation of this inflammatory response is acute but progressive neck pain. It is a rare but important cause of neck pain that should be considered on a differential diagnosis when distinguishing between life-threatening conditions and non-life-threatening causes of neck pain. CASE REPORT: A 51-year-old woman presented to the emergency department (ED) reporting a mild sore throat that progressed to acute neck pain and stiffness. She also reported fatigue, fever, myalgias, and nausea. In the ED, the patient was tachycardic, hypertensive, and mildly febrile with normal oxygen saturation. Examination revealed meningismus and was negative for lymphadenopathy, oropharyngeal findings, and neurologic deficits. Laboratory studies were significant for leukocytosis. Computed tomography (CT) neck was obtained and was notable for calcification of the superior left longus colli muscle with prevertebral and retropharyngeal space edema along the muscle body. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: ACT of the LCM is a benign, self-limited condition that can present with features overlapping emergent causes of acute neck pain. Correct diagnosis relies on characteristic radiographic findings on CT. Fortunately, patients may be discharged home with a short course of anti-inflammatories and corticosteroids with near-complete resolution of symptoms. Emergency physicians, therefore, can rule out life-threatening causes of neck pain, while also making a definitive diagnosis and initiating effective management for this pathology.


Assuntos
Dor Aguda , Tendinopatia , Feminino , Humanos , Pessoa de Meia-Idade , Cervicalgia/etiologia , Tendinopatia/complicações , Tendinopatia/diagnóstico , Tendinopatia/patologia , Tomografia Computadorizada por Raios X , Febre/diagnóstico , Diagnóstico Diferencial , Rigidez Muscular , Músculos/patologia , Músculos do Pescoço/patologia
17.
Brain Nerve ; 75(6): 749-754, 2023 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-37287358

RESUMO

Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive axial muscle stiffness, central nervous system hyper-excitability, and painful stimulus-sensitive muscle spasms. SPS is classified into classic SPS and SPS variants, including stiff-limb syndrome (SLS) and progressive encephalomyelitis with rigidity and myoclonus (PERM), based on clinical presentation. SPS responds to immunotherapy, and several autoantigens have been identified. Most patients with SPS have high-titers of antibodies against glutamic acid decarboxylase (GAD), the rate-limiting enzyme for the synthesis of γ-aminobutyric acid (GABA), and up to 15% of the patients have antibodies against the glycine receptor α-subunit.


Assuntos
Doenças Autoimunes do Sistema Nervoso , Encefalomielite , Rigidez Muscular Espasmódica , Humanos , Rigidez Muscular Espasmódica/diagnóstico , Rigidez Muscular Espasmódica/terapia , Rigidez Muscular , Sistema Nervoso Central , Glutamato Descarboxilase
18.
Brain ; 146(9): 3705-3718, 2023 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-37018058

RESUMO

Although rigidity is a cardinal motor sign in patients with Parkinson's disease (PD), the instrumental measurement of this clinical phenomenon is largely lacking, and its pathophysiological underpinning remains still unclear. Further advances in the field would require innovative methodological approaches able to measure parkinsonian rigidity objectively, discriminate the different biomechanical sources of muscle tone (neural or visco-elastic components), and finally clarify the contribution to 'objective rigidity' exerted by neurophysiological responses, which have previously been associated with this clinical sign (i.e. the long-latency stretch-induced reflex). Twenty patients with PD (67.3 ± 6.9 years) and 25 age- and sex-matched controls (66.9 ± 7.4 years) were recruited. Rigidity was measured clinically and through a robotic device. Participants underwent robot-assisted wrist extensions at seven different angular velocities randomly applied, when ON therapy. For each value of angular velocity, several biomechanical (i.e. elastic, viscous and neural components) and neurophysiological measures (i.e. short and long-latency reflex and shortening reaction) were synchronously assessed and correlated with the clinical score of rigidity (i.e. Unified Parkinson's Disease Rating Scale-part III, subitems for the upper limb). The biomechanical investigation allowed us to measure 'objective rigidity' in PD and estimate the neuronal source of this phenomenon. In patients, 'objective rigidity' progressively increased along with the rise of angular velocities during robot-assisted wrist extensions. The neurophysiological examination disclosed increased long-latency reflexes, but not short-latency reflexes nor shortening reaction, in PD compared with control subjects. Long-latency reflexes progressively increased according to angular velocities only in patients with PD. Lastly, specific biomechanical and neurophysiological abnormalities correlated with the clinical score of rigidity. 'Objective rigidity' in PD correlates with velocity-dependent abnormal neuronal activity. The observations overall (i.e. the velocity-dependent feature of biomechanical and neurophysiological measures of objective rigidity) would point to a putative subcortical network responsible for 'objective rigidity' in PD, which requires further investigation.


Assuntos
Doença de Parkinson , Humanos , Rigidez Muscular/etiologia , Rigidez Muscular/diagnóstico , Rigidez Muscular/tratamento farmacológico , Reflexo de Estiramento/fisiologia , Reflexo Anormal , Eletromiografia
19.
Am J Emerg Med ; 65: 76-83, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36592564

RESUMO

INTRODUCTION: Acute bacterial meningitis in adults is a rare but serious condition that carries a high rate of morbidity. OBJECTIVE: This review highlights pearls and pitfalls of acute bacterial meningitis in adults, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: Meningitis encompasses a broad spectrum of disease involving inflammation of the meninges and subarachnoid space. It classically presents with fever, nuchal rigidity, and altered mental status, but this triad is not present in all cases. Up to 95% of patients will have at least two of the following four cardinal symptoms: fever, nuchal rigidity, altered mental status, and headache. The most common bacterial etiologies are S. pneumoniae and N. meningitidis. Cerebrospinal fluid testing obtained by lumbar puncture remains the gold standard in diagnosis. Head computed tomography prior to lumbar puncture may not be necessary in most patients. Empiric treatment consists of vancomycin, ceftriaxone, and dexamethasone. Elevated intracranial pressure should be managed using established neurocritical care strategies. CONCLUSION: A better understanding of the pearls and pitfalls of acute bacterial meningitis can assist emergency clinicians in pursuing its timely diagnosis and management.


Assuntos
Meningites Bacterianas , Rigidez Muscular , Adulto , Humanos , Prevalência , Meningites Bacterianas/microbiologia , Ceftriaxona , Cefaleia/etiologia , Streptococcus pneumoniae , Punção Espinal/métodos , Antibacterianos/uso terapêutico
20.
BMJ Support Palliat Care ; 13(e2): e306-e307, 2023 Dec 07.
Artigo em Inglês | MEDLINE | ID: mdl-33952581

RESUMO

Progressive supranuclear palsy is a degenerative neurological condition with a high level of associated motor symptom burden manifesting in poor postural reflexes, bradykinesia, dystonia and stiffness in the body core and neck. In the light of a paucity in literature exploring pain management in neurodegenerative diseases, the below case report describes the use of dantrolene to successfully relieve distressing widespread dystonia and muscle rigidity refractory to non-pharmacological and pharmacotherapy. To our knowledge, this is the first reported case of dantrolene use for the treatment of refractory muscle rigidity pain in neurodegenerative conditions.


Assuntos
Distonia , Doenças Neurodegenerativas , Paralisia Supranuclear Progressiva , Humanos , Paralisia Supranuclear Progressiva/complicações , Paralisia Supranuclear Progressiva/tratamento farmacológico , Paralisia Supranuclear Progressiva/diagnóstico , Rigidez Muscular/tratamento farmacológico , Rigidez Muscular/complicações , Dantroleno/uso terapêutico
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