Autoimmune encephalitis is a recently described pathology as inflammatory disease of the central nervous system, previously characterized as a paraneoplastic syndrome. However, advances in technology have allowed to categorize it as an entity, with an specific physiopathology, and a clinical presentation based on the developed auto-antibody, but, the wide clinical presentation, the insidious development of the symptoms, the mimic with other pathologies and the lack of knowledge of this condition in the emergency room and hospitalization services, does not permit to diagnose this condition, leading to potentially fatal complications. We report the case of a 26-years-old female with autoimmune encephalitis, and a literature review regarding this topic.
Anticuerpos antineuronales
Síndrome de Persona Rígida de Causa Paraneoplásica. Claves Diagnósticas en una Entidad Rara. Stiff Person Syndrome Of Paraneoplastic Cause. Diagnostic Keys In A Rare Entity
Introduction: Paraneoplastic disorders exert their effect through immunological mechanisms and not by direct invasion of the tumor; in this way tumors located outside the nervous system induce the production of antibodies against antigens: on the neu- ronal surface, intracellular or synaptic and affect distant sites from its location to either the central or peripheral nervous system, thus producing various clinical manifestations. One of these syndromes is the Stiff Person syndrome, which, apart from being a rare entity, occurs as a paraneoplastic syndrome in only 5% of cases.
Clinical Case: A 72-year-old male patient, a 2-month evolution that began with painful muscle spasms in the dorsal and lumbar region, evolved to rigidity with posture in axial and appendicular hyperextension, pseudospastic gait, hyperekplexia; due to the suspicion of Stiff Person syndrome a diagnostic work-up was begun finding high levels of Ab anti GAD65 and later a Non- Hodgkin’s Lymphoma. The patient received immunotherapy and cancer treatment.
Conclusion: Paraneoplastic syndromes must be identified according to the clinical phenotype and the determination of an- tineuronal antibodies. High suspicion and rapid diagnosis are necessary to achieve timely treatment and avoid permanent injury.
