Subcutaneous Immunoglobulin Therapy with IgPro20 in Patients with Stiff Person Syndrome and Primary Immunodeficiency Disease

2021 ◽  
pp. 1-5
Author(s):  
Olivia Francis ◽  
Avni Joshi ◽  
Ty Prince ◽  
Guha Krishnaswamy ◽  
Niraj C. Patel
Keyword(s):  
Glutamic Acid Decarboxylase ◽  
Immunoglobulin Therapy ◽  
Primary Immunodeficiency Disease ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Subcutaneous Immunoglobulin ◽  
Gad Antibodies ◽  
Neurologic Disorder ◽  
Muscle Spasms ◽  
Immunodeficiency Disease

Stiff Person Syndrome (SPS), a rare autoimmune neurologic disorder characterized by fluctuating muscle spasms and rigidity, is mediated by autoantibodies to glutamic acid decarboxylase (GAD) antibodies. Symptoms of SPS have been shown to improve after administration of intravenous immunoglobulin (IVIG) however, there is a paucity of information regarding use of SCIg in SPS. Four patients with Stiff Person Syndrome were treated with SCIgPro20 for a period between 31 to 101 months. Most reactions were local and mild. All patients reported improvement in spasticity, and 2 patients reported improvement in seizure frequency. SCIgPro20 was well tolerated in patients with SPS and was associated with improvement in symptoms.

scholarly journals Paraneoplastic Stiff Person Syndrome in Early-Stage Breast Cancer with Positive Anti-Amphiphysin Antibodies

2020 ◽  
Vol 12 (3) ◽  
pp. 339-347
Author(s):  
Vitalie Vacaras ◽  
Enia Eleonora Cucu ◽  
Roxana Radu ◽  
Dafin Fior Muresanu
Keyword(s):  
Breast Cancer ◽  
Breast Tumor ◽  
Early Stage ◽  
Acid Decarboxylase ◽  
Muscle Rigidity ◽  
Stiff Person Syndrome ◽  
Gad Antibodies ◽  
Neurologic Disorder ◽  
Muscle Cramps ◽  
Classic Form

Stiff person syndrome (SPS) is a rare neurologic disorder, characterized by muscle rigidity and spasms. Anti-glutamic acid decarboxylase (anti-GAD) antibodies are associated with the classic form of SPS, while antibodies against amphiphysin are associated with the paraneoplastic form of the disease. We present the case of a patient with paraneoplastic SPS, presenting with muscle cramps of lower extremities that progressed to severe muscle rigidity and spasms, associated with a right breast tumor and positive anti-amphiphysin antibodies. Paraneoplastic SPS is a rare neurological disorder, challenging for the physicians both to diagnose and treat.

scholarly journals Stiff Person Syndrome Associated with Compartment Syndrome

2019 ◽  
Vol 11 (2) ◽  
pp. 217-221
Author(s):  
Kok Pin Yong ◽  
Yew Long Lo
Keyword(s):  
Case Report ◽  
Glutamic Acid ◽  
Compartment Syndrome ◽  
Glutamic Acid Decarboxylase ◽  
Neurological Disorder ◽  
Medical Emergency ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
First Case ◽  
Muscle Spasms

Stiff person syndrome (SPS) is a rare and disabling neurological disorder of autoimmune origin, characterized by progressive stiffness and muscle spasms affecting the axial and limb muscles, most frequently associated with antibodies against glutamic acid decarboxylase. We describe a patient who presented initially with compartment syndrome and was later diagnosed with SPS.This is the first case report of SPS possibly presenting initially with compartment syndrome. This case illustrates the importance of recognizing that patients with SPS may present with varied manifestations, including compartment syndrome, which by itself is a medical emergency.

scholarly journals Neurological disorders associated with glutamic acid decarboxylase antibodies: a Brazilian series

2012 ◽  
Vol 70 (9) ◽  
pp. 657-661 ◽  
Author(s):  
Maurício Fernandes ◽  
Renato P. Munhoz ◽  
Paulo Eduardo Mestrinelli Carrilho ◽  
Walter O. Arruda ◽  
Paulo J. Lorenzoni ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Glutamic Acid ◽  
Cerebellar Ataxia ◽  
Glutamic Acid Decarboxylase ◽  
Neurological Disorders ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Gad Antibodies ◽  
Glutamic Acid Decarboxylase Antibodies

Neurological disorders associated with glutamic acid decarboxylase (GAD) antibodies are rare pleomorphic diseases of uncertain cause, of which stiff-person syndrome (SPS) is the best-known. Here, we described nine consecutive cases of neurological disorders associated with anti-GAD, including nine patients with SPS and three cases with cerebellar ataxia. Additionally, four had hypothyroidism, three epilepsy, two diabetes mellitus and two axial myoclonus.

scholarly journals Improvement of stiff-person syndrome symptoms in pregnancy

2020 ◽  
Vol 7 (3) ◽  
pp. e684 ◽  
Author(s):  
Megan E. Esch ◽  
Scott D. Newsome
Keyword(s):  
Glutamic Acid ◽  
Clinical Improvement ◽  
Glutamic Acid Decarboxylase ◽  
Clinical Symptoms ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Treatment Changes ◽  
Gad65 Antibody ◽  
Insight Into ◽  
In Pregnancy

ObjectiveTo describe 2 cases from a single academic institution of improvement in stiff-person syndrome (SPS) symptoms during pregnancy and to review the clinical outcomes of SPS in 6 additional pregnancies described in the literature.MethodsEvaluation of clinical symptoms and treatment changes of disease state during pregnancy.ResultsSeven patients with 9 pregnancies are described in women with a diagnosis of SPS. Six of 7 (86%) women were positive for glutamic acid decarboxylase (GAD65) antibody. In 5 of 9 (56%) pregnancies, symptomatic medications (antispasmodics) were significantly reduced with stabilization or improvement in symptoms through pregnancy. Nine live, healthy pregnancies resulted. All 7 (100%) women experienced worsening of symptoms after the birth of their children, and symptomatic therapies were resumed and/or increased.ConclusionsThe immune pathogenesis of SPS continues to be explored. Immunomodulatory shifts during pregnancy may influence changes of clinical SPS symptoms and provide insight into the unique pathogenesis of SPS. Some women with SPS may be able to reduce symptomatic medications related to clinical improvement during pregnancy. Women with SPS may safely carry pregnancies to term, delivering healthy and unaffected babies.

scholarly journals Autoimmune musicogenic epilepsy associated with anti‐glutamic acid decarboxylase antibodies and Stiff‐person syndrome

2019 ◽  
Vol 8 (1) ◽  
pp. 61-64 ◽  
Author(s):  
Joana Jesus‐Ribeiro ◽  
Alireza Bozorgi ◽  
Modhi Alkhaldi ◽  
Mahmoud Shaqfeh ◽  
Guadalupe Fernandez‐Baca Vaca ◽  
...  
Keyword(s):  
Glutamic Acid ◽  
Glutamic Acid Decarboxylase ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Glutamic Acid Decarboxylase Antibodies ◽  
Musicogenic Epilepsy

Case of Bickerstaff encephalitis with positive glutamic acid decarboxylase antibodies

2020 ◽  
Vol 13 (6) ◽  
pp. e234814 ◽  
Author(s):  
Hussnain Javaid ◽  
Urwa Ejaz ◽  
Zbigniew Slowinski
Keyword(s):  
Glutamic Acid ◽  
Glutamic Acid Decarboxylase ◽  
Central Nervous System Involvement ◽  
Intravenous Immunoglobulins ◽  
Young Female ◽  
Good Prognosis ◽  
Acid Decarboxylase ◽  
Gad Antibodies ◽  
Fisher Syndrome ◽  
Delayed Recovery

Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory demyelinating condition, which is similar to Miller-Fisher syndrome (MFS). Ophthalmoplegia and ataxia are common to these syndromes but unlike MFS, BBE is also characterised by central nervous system involvement, most commonly in the form of altered consciousness. BBE usually has a very good prognosis. We present a case of a young female with BBE. Unlike the majority of BBE patients, she (1) was negative for anti-GBQ1b antibodies but positive for glutamic acid decarboxylase (GAD) antibodies and borderline positive for voltage-gated calcium channel antibodies and (2) had a delayed recovery post treatment with intravenous immunoglobulins and plasma exchange. We contemplate a potential role for GAD antibodies as a marker for longer recovery time in patients with BBE.

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