Novel use of dual immunomodulation for treating stiff-person syndrome, cerebellar variant.
نویسندگان
چکیده
Stiff-person syndrome is an autoimmune syndrome defined by muscle spasms and rigidity of proximal and axial muscles. Glutamic acid decarboxylase is the autoantigen found in 60%–80% of stiff person syndrome patients. Stiff person syndrome, cerebellar variant was characterized in a subpopulation of patients (5 of 38) with stiff person syndrome and concomitant cerebellar dysfunction and confirmed in another case series (3 of 61). In both case series, increased intrathecal synthesis of anti–glutamic acid decarboxylase antibodies was found in stiff person syndrome, cerebellar variant patients. Gamma-aminobutyric acid agonists such as diazepam and immunotherapies improved their stiffness but not cerebellar dysfunction. In this case report we evaluate a therapy using rituximab and cyclophosphamide; this dual immunomodulation improved both stiffness and cerebellar function in our patient.
منابع مشابه
Effect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac disease.
BACKGROUND Cerebellar syndrome and small fiber neuropathy may complicate celiac disease (CD) and may be resistant to a strict gluten-free diet. METHODS Case series. RESULTS We report three patients with biopsy-proven CD who developed cerebellar ataxia and neuropathic pain despite strict adherence to a gluten-free diet. A small fiber neuropathy was suggested by skin biopsy findings in two pa...
متن کاملAnti-GAD positive stiff-person syndrome and cerebellar ataxia: two treatable conditions that clinicians should be aware of.
متن کامل
P159: New Findings in The Diagnosis of Autoimmune Diseases of the Nervous System
The antibody of the nervous system is called antibodies that the body makes to the nervous system cells. These antibodies are also very diverse based on the complexity and diversity of the nervous system, and therefore their detection is also associated with particular challenges. Generally, autoimmune diseases are on the rise. The diagnostic techniques for autoimmune diseases are also in progr...
متن کاملRecent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients
Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic vari...
متن کاملStiff person case misdiagnosed as conversion disorder: A case report
Background: Stiff person syndrome (SPS) is a rare neurological disease resulting in stiffness and spasm of muscles. It initially affects the axial muscles and then spread to limb muscles. Emotional stress exacerbated the symptoms and signs of the disease. The pathophysiology of the disease is caused by the decreased level of the glutamic acid decarboxylase (GAD) activity due to an autoantibody ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Movement disorders : official journal of the Movement Disorder Society
دوره 28 13 شماره
صفحات -
تاریخ انتشار 2013