ABSTRACT
BACKGROUND: Autoimmune autonomic ganglionopathy is a rare, immune-mediated disorder associated with anti-ganglionic α3-subunit nicotinic acetylcholine receptor (anti-α3gAChR) antibodies, which bind to acetylcholine receptor in autonomic ganglia (parasympathetic and sympathetic) leading to autonomic failure. This disorder is mostly associated with viral infections, but it can also be associated with systemic malignancies. Here, we report the case of a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer. METHOD: Case report. RESULTS: A 47-year-old man, without medical history of interest, stated to the emergency department for progressive blurry vision with eye and mouth dryness, constipation, and dizziness upon standing for the last 2 weeks. Orthostatic hypotension was demonstrated by a drop in 13.3 mmHg mean blood pressure (BP) from supine (100/60 mmHg) to 45° reclining sitting position (80/50 mmHg). Blood tests, chest X-ray, brain MRI, and electroneuronography were unremarkable. Electrochemical skin conductance was reduced. Serological examination was positive for anti-α3gAChR antibodies. A full-body CT scan revealed a bladder tumor, which was treated by transurethral bladder resection. The pathologic study demonstrated a low-grade non-muscle-invasive bladder urothelial carcinoma. After tumor resection, and treatment with intravenous immunoglobulins and corticoids, a gradually improvement was observed. Today, the patient remains asymptomatic. CONCLUSION: Subacute panautonomic failure can be the first symptom for systemic malignancies. This case reports a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer. This case highlights the importance of a systemic study to rule out the presence of cancer when autoimmune autonomic ganglionopathy is present.
Subject(s)
Autoimmune Diseases of the Nervous System , Autoimmune Diseases , Autonomic Nervous System Diseases , Carcinoma, Transitional Cell , Peripheral Nervous System Diseases , Urinary Bladder Neoplasms , Autoantibodies , Autoimmune Diseases/complications , Autoimmune Diseases/pathology , Autoimmune Diseases of the Nervous System/complications , Autoimmune Diseases of the Nervous System/diagnosis , Autonomic Nervous System Diseases/complications , Autonomic Nervous System Diseases/etiology , Carcinoma, Transitional Cell/complications , Carcinoma, Transitional Cell/pathology , Ganglia, Autonomic/pathology , Humans , Male , Middle Aged , Peripheral Nervous System Diseases/complications , Urinary Bladder Neoplasms/complications , Urinary Bladder Neoplasms/pathologyABSTRACT
Cerebellar cognitive affective syndrome (CCAS) is characterized by alterations at the cognitive level (dysexecutive syndrome, visuospatial deficit, language ...), associated with affective / emotional changes.
Subject(s)
Cerebellar Diseases , Cognition Disorders , Cerebellar Diseases/complications , Cerebellar Diseases/psychology , Cognition , Cognition Disorders/complications , Humans , Language , SerotoninABSTRACT
El síndrome cognitivo afectivo cerebeloso (SCAC) se caracteriza por alteraciones a nivel cognitivo (síndrome disejecutivo, déficit visuoespacial, lenguaje ), asociado a cambios afectivos/emocionales. Su fisiopatología no es bien conocida y en la actualidad no existe tratamiento específico. Describimos a un hombre de 64 años con cuadro poco frecuente de trastorno cognitivo-conductual tras un infarto en la arteria cerebral media izquierda, dominado por disfunciones ejecutivas, apraxia de predominio oral, atención dividida interrumpida, organización visuoespacial perturbada y anomalías afectivas con una gran apatía, y cuyos síntomas mejoraron con un inhibidor selectivo de la recaptación de la serotonina (ISRS). En ausencia de daño estructural cerebeloso, una tomografía computarizada de perfusión cerebral por emisión de fotón único usando 99mTc-hexametil-propileno-aminoxima (SPECT-HMPAO) mostró una hipoperfusión izquierda frontotemporal y parietoccipital de etiología vascular ya conocida, y una hipoperfusión en hemisferio cerebeloso derecho compatible con fenómeno de diasquisis cruzada. Presumimos que los déficits cognitivos y afectivos están agravados por la interrupción funcional de las interconexiones cerebelosas recíprocas con áreas de asociación cerebral y corteza paralímbica, alterando la contribución del cerebelo al procesamiento y modulación cognitiva y afectiva. En el caso descrito, tanto la situación clínica como las imágenes funcionales de control mejoraron tras tratamiento con ISRS, lo que plantea la posibilidad de que exista conectividad de algunas proyecciones con transmisión serotoninérgica entre el cerebelo y las cortezas de asociación contralateral, y que dicha conectividad disfuncional esté involucrada en la fisiopatología del SCAC.(AU)
Subject(s)
Humans , Health Sciences , Cerebellum , Cognition Disorders , Mood Disorders , Stroke , Selective Serotonin Reuptake Inhibitors , Psychiatry , Neurology , Correspondence as TopicABSTRACT
OBJECTIVES: To analyse the association between aminosalicylate-treated inflammatory bowel disease (IBD) and Parkinson's disease (PD) at population level. DESIGN: Cross-sectional study. SETTING: The study was performed based on electronic drug prescription and dispensation records of the Andalusian Public Health System. PARTICIPANTS: All individuals aged ≥50 years with at least one drug dispensation during December 2014 were identified from the records. PRIMARY AND SECONDARY OUTCOME MEASURES: Groups were formed: 'possible PD' group, including all who received an anti-Parkinson agent; 'possible IBD' group, those treated with mesalazine and/or derivatives (5-aminosalicylic acid (5-ASA)); and 'possible PD and IBD', including those receiving both anti-Parkinson agent and 5-ASA. Prevalence of possible PD was determined among those with possible IBD and among those without this condition. The age-adjusted and sex-adjusted OR was calculated. RESULTS: We recorded 2 020 868 individuals (68±11 years, 56% female), 19 966 were included in possible PD group (75±9 years, 53% female) and 7485 in possible IBD group (64±10 years, 47% female); only 56 were included in both groups (76±8 years, 32% female). The prevalence of possible PD was 0.7% among those with possible IBD and 1% among those without this condition (adjusted OR=0.94; 95% CI 0.72 to 1.23; p=0.657). OR was 0.28 in individuals aged ≤65 years (95% CI 0.10 to 0.74; p=0.01) and 1.17 in older individuals (95% CI 0.89 to 1.54; p=0.257). CONCLUSIONS: Within the limitations of this study, the results suggest a protective role for IBD and/or 5-ASA against PD development, especially among under 65-year olds. Further studies are warranted to explore this association given its scientific and therapeutic implications.