A Case of Myasthenia Gravis and Stiff‐Person Syndrome That Improved After Extended Thymectomy

Respirology Case Reports, Journal Year: 2025, Volume and Issue: 13(3)

Published: March 1, 2025

ABSTRACT In cases of thymoma with autoimmune disease, extended thymectomy may improve disease symptoms and medication effects. Myasthenia gravis (MG) is the most common complication, but Stiff‐Person syndrome (SPS) rare, there are few reports surgery. present case, we performed robot‐assisted on a 74‐year‐old man who had MG SPS. At 6 months after surgery, his SPS improved dose prednisolone been reduced, confirming that surgical treatment contributed to control. Thus far, only two studies have published surgery in conjunction SPS, both documented progression neurological To best our knowledge, this first report resected which were controlled

Language: Английский

---

Ketamine for acute management of refractory stiff person syndrome: a case report

BMC Neurology, Journal Year: 2025, Volume and Issue: 25(1)

Published: April 9, 2025

Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive muscle rigidity and painful spasms. Standard treatments often yield variable responses, particularly in severe, refractory cases. This case report highlights the novel use of ketamine as an effective therapeutic agent for managing acute SPS exacerbations, underscoring its potential second-line treatment patients unresponsive to conventional therapies. A 22-year-old male with SPS, diagnosed via anti-glycine receptor antibodies, presented exacerbation symptoms, including severe stiffness triggered sensory stimuli. Initial management high-dose benzodiazepines, baclofen, intravenous methocarbamol failed provide adequate relief. The patient was subsequently treated ketamine, resulting rapid significant symptom resolution. Despite initial improvement, experienced multiple recurrent flares requiring repeated administration. Over time, proved consistently resolving symptoms when standard were insufficient. patient's complicated anxiety, hypoxia, venous thromboembolism, other comorbidities, highlighting need multidisciplinary approach. illustrates utility providing resolution reducing disease burden during flares. Ketamine's mechanism action, NMDA antagonism enhancement GABAergic signaling, makes it promising adjunct protocols. emphasizes importance individualized, care further research establish ketamine's role long-term SPS.

Language: Английский

---

Proposing Bromo-Epi-Androsterone (BEA) for Stiff Person Syndrome (SPS)

Microorganisms, Journal Year: 2025, Volume and Issue: 13(4), P. 824 - 824

Published: April 5, 2025

SPS is characterized by progressive spasmodic muscular rigidity. thought to be an autoimmune disease with a prominent feature of antibodies against glutamic acid decarboxylase (GAD). GAD responsible for the enzymatic conversion (glutamate) into inhibitory neurotransmitter gamma-aminobutyric (GABA). Reduced GABA activity leads increased excitability in central nervous system, resulting muscle rigidity and spasms characteristic SPS. While rare, anti-GAD seen are also much more common disease, type 1 diabetes (T1D). There evolving research showing that T1D produced response presence mycobacterial heat shock protein 65 (mHSP65), mHSP65 occult infection bacterium, Mycobacterium avium subspecies Paratuberculosis (MAP). Humans broadly exposed MAP food, water, air. linear conformational similarities between epitopes mHSP65. This article proposes infectious trigger Dehydroepiandrosterone (DHEA) principal component steroid metabolome; it plateaus young adults then steadily declines. Bromo-epi-androsterone (BEA) potent synthetic analog DHEA; unlike DHEA, non-androgenic, non-anabolic, effective modulator immune dysregulation. BEA anti-infective agent has been shown benefit infections, including tuberculosis leprosy. With stabilizing capacity as well its anti-mycobacterial properties, there reason believe randomized clinical trial may beneficial

Language: Английский

---

The Effectiveness of Combining Botulinum Toxin Type A and Therapeutic Exercise in Treating Spasticity in a Patient with Complicated Stiff-Person Syndrome: A Case Report

Diseases, Journal Year: 2024, Volume and Issue: 12(6), P. 128 - 128

Published: June 17, 2024

Stiff-person syndrome is rare and disabling autoimmune condition that most frequently affects women, with no real predisposition by race. Diagnosis often arduous, which why patients concomitantly suffer from anxiety depression. To date, drug therapy based on the use of benzodiazepines, barbiturates, baclofen. Refractory cases are treated intravenous immunoglobulin, plasmapheresis, B lymphocyte depletion rituximab, even implantation intrathecal baclofen devices. Botulinum toxin injection used, if it still has an unclear role in literature. Our case report aims to demonstrate efficacy a combined treatment botulinum therapeutic exercise 65-year-old patient biceps brachii muscle hypertonia diffuse spasms axial musculature, using rating scales such as Numeric Rating Scale (NRS) Modified Ashworth (MAS), joint range motion (ROM) measurement, dynamic stiffness mensuration, performed MyotonPro®. All assessments were conducted at first evaluation (T0), soon after botulin (T1), three months (T2), six (T3), eight (T4). The demonstrated benefits for more than 6 side effects. had excellent result our patient.

Language: Английский

---

Core diagnostic features of stiff person syndrome: insights from a case-control study

Journal of Neurology, Journal Year: 2025, Volume and Issue: 272(5)

Published: May 1, 2025

Stiff person syndrome spectrum disorders (SPSD) are rare, disabling neuroimmunological conditions with no consensus diagnostic criteria, making diagnosis challenging. Misdiagnosis often occurs due to the limited awareness of atypical symptoms and presentations. This study aimed identify key clinical paraclinical features most predictive classic SPS SPS-plus misdiagnosis patterns. We conducted a retrospective case-control at Johns Hopkins center, analyzing patient data from 1997-2021. A total 154 SPS, 45 SPS-plus, 66 control patients (evaluated for SPSD but given an alternative diagnosis) were included. Clinical assessments, autoantibody testing, electromyography (EMG), other studies reviewed. Sensitivity, specificity, odds ratios calculated, logistic regression identifying strongest indicators phenotypes. Torso/lower extremity symptoms, hypersensitivity triggers, paravertebral stiffness, gait dysfunction common in both Classic was specifically associated high-titer GAD65 antibodies (98%), cerebrospinal fluid positivity (100%), characteristic EMG abnormalities (> 90%), hyperlordosis (87%). specificity highest cerebellar (98.5%) brainstem (100%) signs/symptoms. High-titer independent factor presenting upper extremity, brainstem, or involvement. Recognizing patterns may help clinicians make accurate timely diagnoses SPSD. could prevent misdiagnosis/overdiagnosis, ensure treatment, assure appropriate populations included future interventional trials. Further needed validate these findings refine criteria.

Language: Английский

---

Hematopoietic Stem Cell Transplantation for the Treatment of Autoimmune Neurological Diseases: An Update

Bioengineering, Journal Year: 2023, Volume and Issue: 10(2), P. 176 - 176

Published: Jan. 29, 2023

Over the last two decades, haematopoietic stem cell transplantation (HSCT) has been explored as a potential therapeutic strategy for autoimmune diseases refractory to conventional treatments, including neurological disorders. Although both autologous (AHSCT) and allogeneic HSCT (allo-HSCT) were investigated, AHSCT was preferentially developed due more favourable safety profile compared allo-HSCT. Multiple sclerosis (MS) represents most frequent indication AHSCT, but increasing evidence on effectiveness of transplant in other is emerging, although with risk-benefit ratio overall uncertain than MS. In present work, rationale use experimental models that prompted its clinical application will be briefly covered. Case series prospective studies exploring MS discussed, covering rare disorders such myasthenia gravis, myopathies, stiff-person syndrome. Finally, an updated summary ongoing future focusing this issue provided.

Language: Английский

---

Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

Biomedicines, Journal Year: 2023, Volume and Issue: 11(9), P. 2500 - 2500

Published: Sept. 10, 2023

Stiff person spectrum disorders (SPSD) are paradigm autoimmune movement characterized by stiffness, spasms and hyperekplexia. Though rare, SPSD represent a not-to-miss diagnosis because of the associated disease burden treatment implications. After decades as an enigmatic orphan disease, major advances in our understanding evolving diseases have been made along with identification multiple autoantibodies. However, most important recent developments relate to recognition wider affection, beyond classic core motor symptoms, further insights into immunomodulatory symptomatic therapies. In this review, we summarize literature on clinical paraclinical spectrum, current pathophysiological understanding, well possibly future therapeutic strategies.

Language: Английский

---

Anti-Idiotypic mRNA Vaccine to Treat Autoimmune Disorders

Vaccines, Journal Year: 2023, Volume and Issue: 12(1), P. 9 - 9

Published: Dec. 21, 2023

The 80+ existing autoimmune disorders (ADs) affect billions with little prevention or treatment options, except for temporary symptomatic management, leading to enormous human suffering and a monumental financial burden. autoantibodies formed in most ADs have been identified, allowing the development of novel anti-idiotypic antibodies mute using vaccines. Nucleoside vaccines successfully tested as antigen-specific immunotherapies (ASI), mRNA technology offering multi-epitope targeting multiple autoantibodies. This paper proposes produce broad effectiveness preventing treating them. delves into state-of-the-art design strategies used develop ASIs by selecting appropriate T cell B epitopes generate antibodies. low cost fast make this affordable global control ADs.

Language: Английский

---

Stiff-person syndrome

Practical Neurology, Journal Year: 2024, Volume and Issue: unknown, P. pn - 003974

Published: Sept. 2, 2024

Stiff-person syndrome (SPS) is an autoimmune disease associated mainly with antibodies to glutamic acid decarboxylase (GAD) or glycine, characterised by intermittent painful spasms, stiffness and rigidity of the proximal truncal muscles. Neuro-ophthalmological gastrointestinal symptoms also occur. The are caused neuronal excitability due impaired inhibitory (gamma amino butyric [GABA] glycine) neurotransmission. SPS part a larger spectrum GAD antibody-spectrum disorders, which overlaps epilepsy, cerebellar ataxia, myoclonus, progressive encephalomyelitis, myoclonus (PERM) limbic encephalitis. PERM often against glycine receptor. Some cases paraneoplastic. Diagnostic delay irreversible disability, therefore, clinicians need high degree clinical suspicion make earlier diagnosis. This review updates various presentations that should raise its related conditions includes diagnostic algorithm treatment strategies including immunotherapy GABA-ergic drugs.

Language: Английский

---

Differential Diagnosis of Suspected Multiple Sclerosis in Pediatric and Late-Onset Populations

JAMA Neurology, Journal Year: 2024, Volume and Issue: 81(11), P. 1210 - 1210

Published: Sept. 16, 2024

While the typical onset of multiple sclerosis (MS) occurs in early adulthood, 2% to 10% cases initially present prior age 18 years, and approximately 5% after 50 years. Guidance on approaches differential diagnosis suspected MS specific these 2 groups is needed.

Language: Английский

---