Read by QxMD icon Read

Stiff person syndrome

Karolina Popławska-Domaszewicz, Jolanta Florczak-Wyspiańska, Wojciech Kozubski, Sławomir Michalak
Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Two types of antibodies can be distinguished: antibodies against nuclear and cytoplasmic neuronal antigens (anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-CV2/CRMP5, anti-Gephrin, and anti-GABATRAP) and antibodies recently identified against cell surface and synaptic proteins (anti-NMDAR, anti-LGI1, and anti-Caspr2)...
March 21, 2018: Reviews in the Neurosciences
Justin Ralph Abbatemarco, Mary Alissa Willis, Robert G Wilson, Sean J Nagel, Andre G Machado, Francois A Bethoux
OBJECTIVES: Stiff-person syndrome (SPS) is associated with axial rigidity superimposed on sustained muscle spasms. These symptoms commonly interfere with the performance of activities of daily living including ambulation. This retrospective case series evaluates the outcomes of screening tests and chronic infusion of intrathecal baclofen (ITB) in patients diagnosed with SPS treated in our spasticity clinic. MATERIALS AND METHODS: Patients were identified from an institutional review board-approved clinical registry of ITB therapy...
March 13, 2018: Neuromodulation: Journal of the International Neuromodulation Society
Shannon R Hinson, A Sebastian Lopez-Chiriboga, James H Bower, Joseph Y Matsumoto, Anhar Hassan, Eati Basal, Vanda A Lennon, Sean J Pittock, Andrew McKeon
Background: Glycine receptor alpha-1 subunit (GlyRα1)-immunoglobulin G (IgG) is diagnostic of stiff-person syndrome (SPS) spectrum but has been reported detectable in other neurologic diseases for which significance is less certain. Methods: To assess GlyRα1-IgGs as biomarkers of SPS spectrum among patients and controls, specimens were tested using cell-based assays (binding [4°C] and modulating [antigen endocytosing, 37°C]). Medical records of seropositive patients were reviewed...
March 2018: Neurology® Neuroimmunology & Neuroinflammation
Jason Schend, Dave McGarry, John Johnson, Robert Hostoffer
No abstract text is available yet for this article.
January 30, 2018: Annals of Allergy, Asthma & Immunology
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
H-M Meinck, B Balint
The identification of new variants of the stiff man syndrome (SMS) and of new, probably pathogenic neuronal autoantibodies has led to the concept of stiff man (or person) spectrum disorders (SPSD). This is an expanding group of rare chronic autoimmune inflammatory diseases of the central nervous system (CNS) that have in common the main symptoms of fluctuating rigidity and spasms with pronounced stimulus sensitivity. These core symptoms are mandatory and can be accompanied by a wide variety of other neurological signs...
February 2018: Der Nervenarzt
Aude Belbezier, Bastien Joubert, Gonzalo Montero-Martin, Marcelo Fernandez-Vina, Nicole Fabien, Véronique Rogemond, Emmanuel Mignot, Jérôme Honnorat
Objective: Neurologic autoimmune syndromes associated with anti-glutamate acid decarboxylase 65 antibodies (GAD65-Abs) are rare and mostly sporadic. Methods: We describe a niece and her aunt with GAD65-Abs neurologic syndromes. High-resolution HLA typing of Class I and Class II alleles was performed using next-generation sequencing. Results: The proband had cerebellar ataxia and probable limbic encephalitis features, whereas her niece had stiff-person syndrome...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
Faruk Incecik, Ozlem M Herguner, Seyda Besen, Mustafa Yılmaz
Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients including stiff person syndrome, cerebellar ataxia, refractory epilepsy, limbic and extralimbic encephalitis. GAD antibodies-related limbic encephalitis cases are well described; reports of extralimbic involvement are limited. We describe four cases of GAD antibody-related autoimmune encephalitis. Three of them had extralimbic involvement and only one had limbic encephalitis.
January 18, 2018: Acta Neurologica Belgica
Hideto Nakajima, Yoshitsugu Nakamura, Yuiko Inaba, Chiharu Tsutsumi, Kiichi Unoda, Takafumi Hosokawa, Fumiharu Kimura, Toshiaki Hanafusa, Masamichi Date, Haruko Kitaoka
To determine clinical features of neurologic disorders associated with anti-glutamic acid decarboxylase antibodies (anti-GAD-Ab), we examined titers and time-dependent changes of anti-GAD-Ab. Six patients, stiff person syndrome (2), cerebellar ataxia (1), limbic encephalitis (1), epilepsy (1), brainstem encephalitis (1), were compared with 87 type I diabetes mellitus (T1DM) patients without neurologic disorders. Anti-GAD-Ab titers and index were higher in neurologic disorders than in T1DM, suggesting intrathecal antibody synthesis...
January 9, 2018: Journal of Neuroimmunology
Xiao-Zhong Jing, Dan Zhu, Ya-Qian Zhang, Ming Dong
No abstract text is available yet for this article.
January 16, 2018: Neurology
Toshio Fukutake
Apart from the term 'involuntary movements', the term 'movement disorders' encompasses not only classical hyperkinesias but also hypokinesias including catalepsy or apraxia. It enables us to understand abnormal movements by their phenomena instead of by their localization. To advance the grasping of movement disorders, we discuss the clinical and pathophysiological features of abnormal movements in catatonia/catalepsy, anti-NMDAR encephalitis, paroxysmal dyskinesias, stiff person/leg syndrome, corticobasal degeneration/syndrome, and hysteria...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
Nathan E Esplin, John W Stelzer, Timothy B Legare, Sayed K Ali
Background: Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle rigidity. It is a disorder of reduced GABA activity leading to increased muscle tone and often painful spasms. It generally presents in the axial musculature but rarely can involve only one limb, typically a lower extremity. In rare cases it can be paraneoplastic which generally resolves on treatment of the underlying neoplasm. Case Report: A 46-year-old male with a history of Hodgkin's Lymphoma in remission presented with left upper extremity pain secondary to a diagnosis of Stiff Person Syndrome limited to his left upper extremity...
2017: Case Reports in Neurological Medicine
Saeed Razmeh, Amir Hasan Habibi, Farzad Sina, Elham Alizadeh, Monireh Eslami
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 against GAD that decreases the level of gamma-aminobutyric acid (GABA). In this paper, we present a case of atypical presentation of SPS with lower limb stiffness misdiagnosed as conversion disorder...
2017: Caspian Journal of Internal Medicine
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
A Llewellyn, C S McCabe, Y Hibberd, P White, L Davies, J Marinus, R G S M Perez, I Thomassen, F Brunner, C Sontheim, F Birklein, T Schlereth, A Goebel, R Haigh, R Connett, C Maihöfner, L Knudsen, R N Harden, A Zyluk, D Shulman, H Small, F Gobeil, P Moskovitz
BACKGROUND: Complex Regional Pain Syndrome (CRPS) symptoms can significantly differ between patients, fluctuate over time, disappear or persist. This leads to problems in defining recovery and in evaluating the efficacy of therapeutic interventions. OBJECTIVES: To define recovery from the patients' perspective and better understand their priorities for treatment approaches. METHODS: Establishing an international consortium, we used a 2-Round Delphi-based study in eight countries across Europe and North America...
March 2018: European Journal of Pain: EJP
Stoyan Popkirov, Seena Sebastian, Fatme Seval Ismail, Jörg Wellmer
Antibodies against glutamic acid decarboxylase (GAD) are a hallmark of type 1 diabetes and its late-onset variant, latent autoimmune diabetes of the adult (LADA).(1) While cerebrovascular disease and dementia are common causes of cognitive dysfunction and neurological deficits in diabetic patients,(2,3) rare autoimmune disorders of the central nervous system such as stiff-person syndrom or limbic encephalits can also occur on the basis of shared GAD autoimmunity.(4,5).
November 20, 2017: Journal of Diabetes
Stephanie Yu Shan Yong, Jin Yao Teo, Kok Pin Yong, Brian K P Goh
No abstract text is available yet for this article.
January 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Thien Thien Lim
PURPOSE OF REVIEW: To provide an overview of paraneoplastic autoimmune disorders presenting with various movement disorders. RECENT FINDINGS: The spectrum of paraneoplastic autoimmune disorders has been expanding with the discovery of new antibodies against cell surface and intracellular antigens. Many of these paraneoplastic autoimmune disorders manifest as a form of movement disorder. With the discovery of new neuronal antibodies, an increasing number of idiopathic or neurodegenerative movement disorders are now being reclassified as immune-mediated movement disorders...
November 2017: Parkinsonism & related Disorders
Bettina Balint, Angela Vincent, Hans-Michael Meinck, Sarosh R Irani, Kailash P Bhatia
Movement disorders are a prominent and common feature in many autoantibody-associated neurological diseases, a group of potentially treatable conditions that can mimic infectious, metabolic or neurodegenerative disease. Certain movement disorders are likely to associate with certain autoantibodies; for example, the characteristic dyskinesias, chorea and dystonia associated with NMDAR antibodies, stiff person spectrum disorders with GAD, glycine receptor, amphiphysin or DPPX antibodies, specific paroxysmal dystonias with LGI1 antibodies, and cerebellar ataxia with various anti-neuronal antibodies...
January 1, 2018: Brain: a Journal of Neurology
Yoshimitsu Maki, Hiroshi Takashima
In recent years, incidence of autoimmune encephalopathies has increased. The diagnosis of the severe form of autoimmune encephalopathy is not difficult; however, milder forms can be misdiagnosed as general encephalopathies. We often treat Hashimoto's encephalopathy, which has diverse clinical symptoms and is often misdiagnosed as a psychosomatic disease. We have found that the neurological findings and symptoms of patients with Hashimoto's encephalopathy are similar to those of psychogenic diseases, such as giveway weakness and atypical sensory disorder...
October 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"