Consilium MedicumConsilium Medicum2075-17532542-2170Consilium Medicum9489110.26442/2075-1753_19.9.65-68Research ArticleThe syndrome of a rigid person with myoclonus and disautonomy: a description of the caseSerdyukA. Vmailaserdyuk@gmail.comKovrazhkinaE. Aelekov@yandex.ruN.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation15092017199656828122021Copyright © 2017, Consilium Medicum2017The syndrome of a rigid person is a rare autoimmune disease characterized by progressive muscular rigidity, walking disorders and painful spasms in axial muscles and limbs provoked by external stimuli. The syndrome has a different clinical phenomenology, it can be manifested by myoclonus, vegetative and emotional disorders, combined with epilepsy and dystonia, and neuro-ophthalmologic manifestations. The pathogenesis of the syndrome is associated with a violation of GABAergic transmission as a result of the interaction of specific antibodies with glutamate decarboxylase (glutamate decarboxylase), an enzyme involved in the synthesis of y-aminobutyric acid. Taking into account the pathogenesis in the treatment of this disease, GABAergic drugs (diazepam in high doses, etc.) and immunosuppressive therapy are used. We give our own observation of a patient with an atypically occurring syndrome of a rigid person.syndrome of rigid manglutamate decarboxylaseimmunosuppressive therapydisautonomyclinical caseсиндром ригидного человекаглутаматдекарбоксилазаиммуносупрессивная терапиядизавтономияклинический случай[Rakocevic G, Floeter M.K. Autoimmune stiff person syndrome and related myelopaties: understanding of electrophysiological and immunological processes. Muscle Nerve 2012; 45 (5): 623-34.][Meinck H.M, Ricker K, Hulser P.J, Solimena M. Stiff man syndrome: neurophysiological findings in eight patients. J Neurol 1995; 242 (3): 134-42.][Shaw P.J. Stiff-man syndrome and its variants. Lancet 1999; 353 (9147): 86-7.][Brown P., Marsden C.D. The stiff man and stiff man plus syndromes. J Neurol 1999; 246 (8): 648-52.][Saiz A, Blanco Y, Sabater L. et al. Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association. Brain 2008; 131 (Pt. 10): 2553-63.][Tohid H. Antiglutamic acid decarboxylase antibody positive neurological syndromes. Neurosci (Riyadh) 2016; 21 (3): 215-22.][Sarva H, Deik A, Ullah A, Severt W.L. Clinical spectrum of stiff person syndrome: areview of recent reports. Tremor Other Hyperkinet Mov (NY) 2016; 6: 340.][Краснов М.Ю., Павлов Э.В., Ершова М.В. и др. Спектр неврологических синдромов, ассоциированных с антителами к глутамат-декарбоксилазе. Анналы неврологии. 2015; 4: 37-41.][Keon A, Tracy J.A. GAD65 neurological autoimmunity. Muscle Nerve 2017.][Ali F, Rowley M., Jayakrishnan B et al. Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: Protean additions to the autoimmune central neuropathies. J Autoimmun 2011; 37: 79-87.][Holmoy T., Geis C. The immunological basis for treatment of stiff person syndrome. J Neuroimmunol 2011; 231: 55-60.][McKeon A., Martinez-Hernandez E., Lancaster E. et al. Glycine receptor autoimmune spectrum with stiff-man syndrome phenotype. JAMA Neurol 2011; 70 (1): 44-50.][Carvajal-Gonzalez A, Leite M.I, Waters P. et al. Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. Brain 2014; 137 (8): 2178-92.][Dalakas M.C. Stiff person syndrome: advances in pathogenesis and therapeutic interventions. Curr Treat Options Neurol 2009; 11: 102-10.]