RESUMO

Stiff-person syndrome is characterized by persistent muscle spasms, involving agonist and antagonist muscles simultaneously, starting in the lower limbs and trunk. It tends to occur in the fourth to sixth decade of life, presenting with intermittent spasms that later become continuous and usually painful. Minor sensory stimuli, such as noise or light touch, precipitate severe spasms. Spasms do not occur during sleep and only rarely involve cranial muscles. We present a case that for two years was diagnosed and treated as a conversion disorder associated with depression. After two years she was admitted to another hospital with an unmistakable picture of stiff-person syndrome with hypertrophy and rigidity of lower limb muscles, compatible electrophysiology and positive anti-GAD antibodies. She had autoimmune hypothyroidism, that should have raised the suspicion of stiff-person syndrome earlier. She responded to intravenous immunoglobulin and mycophenolate mofetil and and to tranquilizers that have muscle relaxant properties.

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RESUMO

Introducción: Los trastornos somatomorfos (TS) y conversivos (TC) han aumentado su incidencia durante las dos últimas décadas en niños y adolescentes, aunque ambos son habitualmente subdiagnosticados. Objetivo: Efectuar una puesta al día de la evidencia sobre TS y TC en niños y adolescentes. Método: Se realizó una búsqueda bibliográfica mediante las bases de datos Medline/PubMed, SciELO y textos especializados. Resultados: Existe una alta intercurrencia con trastornos afectivos y ansiosos y se han identificado algunos factores de riesgo, especialmente mayor vulnerabilidad al estrés, disfunción familiar, sobreprotección parental y limitada mentalización de los síntomas físicos (alexitimia), que también perpetuan la sintomatología. Desde la perspectiva etiopatogénica se han invocado hipótesis psicodinámicas, psicosomáticas, traumático-ambientales y biológicas. Los pacientes con TS y TC frecuentemente son mujeres, presentan rasgos más introvertidos de personalidad, más eventos vitales estresantes y défictis ejecutivos. Las convulsiones psicógenas no epilépticas se relacionan con más comorbilidades psiquiátricas y peor pronóstico. Las psicoterapias cognitivo-conductual y la hipnoterapia-psicodinámica combinadas con fármacos inhibidores de la recaptura de serotonina han demostrado ser eficaces en el tratamiento. Conclusiones: Los TS y TC en poblaciones infanto juveniles se observan en contextos médicos y psiquiátricos. Su reconocimiento es difícil debido a su etiología multifactorial, la falta de consenso diagnóstico yla escasa experiencia clínica, implicando un verdadero desafío terapéutico, por lo que es indispensable realizar futuras investigaciones en este tópico. Su manejo en la atención primaria parece apropiado, pero en casos más complejos es necesaria su derivación al especialista.

Background: Somatoform (SD) and conversion (CD) disorders have increased their incidence during the last two decades in children and adolescents, although both are currently underdiagnosed. Objective: To review the evidence on SD and CD in children and adolescents. Method: A review ofspecialised textbooks and the available literature in Medline/PubMed and SciELO was made. Results: There is a high intercurrence with affective and anxiety disorders and some riskfactors have been identified, especially higher vulnerability to stress, family disfunction, parental overprotection, and limited mentalization of physical symptoms (alexithymia), which at the same time, perpetuate the symptomatology. From an ethiopathogenicperspective, psychodynamic, psychosomatic, traumatic-environmental and biological hypothesis have been invoked. Patients with SD and CD are frequently females, show more introversive personality features, more stressful life events and executive deficits. Psychogenic non-epileptic seizures are related with more psychiatric co-morbidities and worse outcome. Cognitive-behavioural psychotherapy and psychodynamic-hypnotherapy combined with selective serotonin reuptake inhibitors have been effective. Conclusions: SD and CD in child and adolescent populations have been observed in medical and psychiatric contexts. Their recognition is difficult due to their multifactorial ethiology, the lack of diagnosis consensus and the scarce clinical experience, involving a true therapeutic challenge; therefore, it is imperative to carry out future researches on this issue. Their management in primary care seems suitable, but in more complex cases the referral to the specialist is necessary.

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