Anxious Depression and the Stiff-person Plus Syndrome

Čulav-Sumić, Jadranka; Bošnjak, Ivan; Paštar, Zvonimir; Jukić, Vlado

doi:10.1097/wnn.0b013e318185e6d2

Cited by 11 publications

(11 citation statements)

References 42 publications

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“…A single case report described a patient with Stiff-person plus syndrome[15] in whom anxious depression was improved after corticosteroid treatment. Although a few case reports showed that neurological manifestations are along with decrease of anti-GAD antibody titer, no correlation was noted between decrease of anti-GAD antibody titers and the magnitude of clinical response in a controlled study, in which a three-month course of high-dose IVIg demonstrated benefits in SPS patients [16].…”

Section: Discussionmentioning

confidence: 99%

Cognitive decline in a patient with anti-glutamic acid decarboxylase autoimmunity; case report

et al. 2011

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BackgroundGlutamic acid decarboxylase (GAD) is the rate-limiting enzyme for producing γ-aminobutyric acid, and it has been suggested that antibodies against GAD play a role in neurological conditions and type 1 diabetes. However, it is not known whether dementia appears as the sole neurological manifestation associated with anti-GAD antibodies in the central nervous system.Case presentationWe describe the clinical, neuropsychological, and neuroradiological findings of a 73-year-old female with cognitive dysfunction and type 1A diabetes. Observation and neuropsychological studies revealed linguistic problems, short-term memory disturbance, and frontal dysfunction. MRI showed no significant lesion except for confluent small T2-hyperintensity areas localized in the left basal ganglia. 18F-fluorodeoxy glucose-positron emission tomography (FDG-PET) and 123I-N-isopropyl-p-iodoamphetamine-single photon emission computed tomography (IMP-SPECT) studies showed bifrontal hypometabolism and hypoperfusion. Immunomodulating therapy with intravenous high-dose immunoglobulin resulted in no remission of the cognitive symptoms.ConclusionsCognitive dysfunction may develop as an isolated neurological manifestation in association with type 1A diabetes and anti-GAD autoimmunity. A systematic study with extensive neuropsychological assessment is indicated in patients with type 1 diabetes and anti-GAD autoimmunity.

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Section: Discussionmentioning

confidence: 99%

Cognitive decline in a patient with anti-glutamic acid decarboxylase autoimmunity; case report

et al. 2011

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“…The identification of patients with GAD65Ab-associated neurological disorders is hampered by a wide range of mild and non-specific symptoms such as phobias (11, 12), depression (13), and muscle aches and pain during the early stages of the neurological disease. The variability of symptoms (14–16) hinders the correct diagnosis of these conditions and previously used strict diagnosis criteria are not always helpful in clinical practice (17).…”

Section: Introductionmentioning

confidence: 99%

High Titers of Autoantibodies to Glutamate Decarboxylase in Type 1 Diabetes Patients: Epitope Analysis and Inhibition of Enzyme Activity

et al. 2013

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Objective Autoantibodies to glutamate decarboxylase (GAD65Ab) are found in patients with autoimmune neurological disorders and patients with type 1 diabetes. The correct diagnosis of GAD65Ab-associated neurological disorders is often delayed by the variability of symptoms and a lack of diagnostic markers. We hypothesize that the frequency of neurological disorders with high GAD65Ab titers is significantly higher than currently recognized. Methods We analyzed GAD65Ab titer, inhibition of GAD65 enzyme activity, and pattern of GAD65Ab epitopes in a cohort of type 1 diabetes patients (n=100) and correlated our findings with neurological symptoms and diseases. Results Fourty-three percent (43/100) of the patients had detectable GAD65Ab titers (median=400 U/ml, range: 142–250,000U/ml). The GAD65Ab titers in 10 type 1 diabetes patients exceeded the 90th percentile of the cohort (2,000–250,000 U/ml). Sera of these 10 patients were analyzed for their GAD65Ab epitope specificity and their ability to inhibit GAD65 enzyme activity in vitro. GAD65Ab of five patients inhibited the enzyme activity significantly (by 34–55%). Three of these patients complained of muscle stiffness and pain, which was documented in two of these patients. Conclusions Based on our findings we suggest that neurological disorders with high GAD65Ab titers are more frequent in type 1 diabetes patients than currently recognized.

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“…El diagnóstico de SPS es con frecuencia difícil y la demora entre el inicio de los síntomas es de 1 a 18 años 1 . El diagnóstico por 2 años de depresión asociada a cuadro conversivo en nuestra paciente ilustra una situación que no es infrecuente en este síndrome 4,7 . El inicio fue insidioso e inespecífico, acompañado principalmente de síntomas mentales con examen neurológico normal o signos sutiles y cambiantes gatillados por las emociones.…”

Section: Discussionunclassified

“…Típicamente los espasmos ocurren en vigilia y desaparecen durante el sueño, lo que indica que se trata de un fenómeno que compromete el sistema nervioso central y no al nervio, unión neuromuscular o músculo 2 . No es infrecuente que el cuadro se presente como parte de un trastorno depresivo ansioso, situación que retarda el diagnóstico y el tratamiento 1,4 . De un punto de vista etiopatogénico, es una enfermedad autoinmune pre-sináptica debido a la producción de anticuerpos contra la decarboxilasa del ácido glutámico (GAD en su acrónimo inglés), una enzima responsable de la síntesis del ácido gama-aminobutírico (GABA en su acrónimo inglés), un neurotransmisor inhibitorio que se encuentra en las neuronas de sistema nervioso central y en las células beta pancreáticas 5 , aunque no parecen ser los únicos causantes de este cuadro 6 .…”

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Síndrome de persona rígida confundido por trastorno conversivo: un error común. Caso clínico

Guajardo¹,

Cea

2019

Rev. méd. Chile

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Stiff-person syndrome mistaken for conversive disorder: a common error 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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Anxious Depression and the Stiff-person Plus Syndrome

Abstract: The authors believe that anxious depression and SPS-plus seen in this patient are the result of the same underlying autoimmune process, together forming a unique syndrome. Anxious and depressive symptoms in SPS can be explained by alterations in GABAergic neurotransmission.

Cited by 11 publications

References 42 publications

Cognitive decline in a patient with anti-glutamic acid decarboxylase autoimmunity; case report

Cognitive decline in a patient with anti-glutamic acid decarboxylase autoimmunity; case report

High Titers of Autoantibodies to Glutamate Decarboxylase in Type 1 Diabetes Patients: Epitope Analysis and Inhibition of Enzyme Activity

Síndrome de persona rígida confundido por trastorno conversivo: un error común. Caso clínico

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