Validation of the Brazilian version of mini-test CASI-S Validação da versão brasileira do mini-teste CASI-S

OBJECTIVE: To determine CASI-S accuracy in the diagnosis of dementia. METHOD: The Cognitive Abilities Screening Instrument - Short Form (CASI-S) was applied in 43 Alzheimer's disease (AD) patients and 74 normal controls. AD diagnosis was based on DSM-IV, NINCDS-ADRDA, and CAMDEX. CASI-S includes: registration, temporal orientation, verbal fluency (4-legged animals in 30s), and recall (3 words). Its maximum score is 33 points. A copy of 2 pentagons was added. RESULTS: ROC curve showed an accuracy of 0.87, with standard error of 0.032, and 95% confidence intervall between 0.795 and 0.925. The cut-off score for cognitive deficit was 23, with sensitivity of 76.7%, specificity 86.5%, positive likelihood ratio (LR) 5.68, and negative LR 0.27. The cut-off score for subjects 70 years or older was 20, with sensitivity of 71.4% and specificity 97.1%. CONCLUSION: CASI-S is a practical test, with high specificity, particularly in individuals above 70 years of age. The adding of the drawing test did not improve its accuracy.OBJETIVO: Determinar a acurácia do CASI-S no diagnóstico de demência. MÉTODO: O CASI-S (Cognitive Abilities Screening Instrument - Short Form) foi aplicado em 43 pacientes com doença de Alzheimer (DA) e 74 controles normais. O diagnóstico de DA baseou-se no DSM-IV, NINCDS-ADRDA e CAMDEX. O CASI-S inclui: registro, orientação temporal, fluência verbal (animais quadrúpedes em 30s), e evocação (3 palavras). O escore máximo é 33 pontos. Foi adicionado um teste de cópia de 2 pentágonos. RESULTADOS: A curva ROC mostrou acurácia de 0,87, com erro padrão de 0,032, e intervalo de confiança de 95% entre 0,795 e 0,925. O ponto de corte para déficit cognitivo foi 23 pontos, com sensibilidade de 76,7%, especificidade de 86,5%, valor preditivo (VP) positivo de 5,68, e VP negativo de 0,27. Para sujeitos com 70 anos ou mais, o ponto de corte foi 20, com sensibilidade de 71,4% e especificidade de 97,1%. CONCLUSION: O CASI-S é um teste prático, com alta especificidade, particularmente em indivíduos com idade acima de 70 anos. O teste de cópia dos pentágonos não melhorou sua acurácia

Vanishing bile duct syndrome related to DILI and Hodgkin lymphoma overlap: A rare and severe case

Vanishing bile duct syndrome is a rare acquired condition, characterized by progressive loss of intrahepatic bile ducts leading to ductopenia and cholestasis. It can be associated with infections, ischemia, drug adverse reactions, neoplasms, autoimmune disease, and allograft rejection. Prognosis is variable and depends on the etiology of bile duct injury. We report the case of a 25-year-old female with cholestatic hepatitis and concomitant intakes of hepatotoxic substances, such as garcinia, field horsetail, and ketoprofen. On suspicion of a drug-induced liver injury, the drugs were promptly withdrawn and ursodeoxycholic acid was started with initial clinical and laboratory improvement, and the patient was discharged from the hospital. One month later, she had a new increase in bilirubin levels and canalicular enzymes, requiring a liver biopsy that showed significant loss of intrahepatic bile ducts, which was compatible with vanishing bile duct syndrome. This was confirmed by using cytokeratin 19 on immunohistochemistry. There was subsequent lymph node enlargement in several chains, and relevant weight loss. Histological analysis of a cervical lymph node revealed nodular sclerosis-subtype classic Hodgkin lymphoma. In this setting, vanishing bile duct syndrome was related to Hodgkin lymphoma and a drug-induced liver injury overlap, leading to progressive cholestasis with a worse prognosis. The patient's response to chemotherapy was poor, requiring biological therapy with brentuximab vedotin. It is crucial for physicians to create a broad differential diagnosis in suspected vanishing bile duct syndrome patients, especially to rule out malignancies

Primitive reflexes and cognitive function Reflexos primitivos e função cognitiva

BACKGROUND: Data on the prevalence of primitive reflexes (PR) in adulthood, their pathological significance and relationship to age and cognition are controversial. OBJECTIVE: To study the relationship between PR and cognition in 30 patients with probable Alzheimer's disease (AD) and 154 control subjects. METHOD: Diagnosis of probable AD was based on DSM-IV, NINCDS-ADRDA, and CAMDEX criteria. Primitive reflexes were quantified from zero (absent) to 1 (mild) or 2 (markedly present). The Cognitive Abilities Screening Instrument - Short Form (CASI-S) was used to evaluate registration, temporal orientation, verbal fluency and recall. A drawing test was added. RESULTS: Most frequent PR among demented and controls were suck (77% and 62%, respectively) and snout (60% and 27%), followed by glabellar (30% and 19%), paratonia (37% and 5%), and palmomental (23% and 5%). None of controls had more than three PR. Frequency of PR tended to increase with age and cognitive deterioration. Grasp and Babinski responses were found only in dementia patients. Primitive reflexes were not correlated with each other, except snout with suck, and snout with glabellar reflex. CONCLUSION: The finding of grasp and Babinski sign, or the presence of more than three primitive signs, particularly the combination of paratonia, snout, suck, and palmomental reflexes strongly suggests brain dysfunction, especially when these signs are marked and accompanied by deficits in orientation, recall, verbal fluency, and constructional praxis.<br>CONTEXTO: A prevalência e significado patológico dos reflexos primitivos (RP) no adulto, bem como sua relação com a idade e a cognição, são questões controversas. OBJETIVO: Estudar a relação entre RP e cognição em 30 pacientes com doença de Alzheimer (DA) e 154 sujeitos controles normais. MÉTODO: O diagnóstico de DA baseou-se nos critérios DSM-IV, NINCDS-ADRDA e CAMDEX. Os RP foram quantificados de 0 (ausente) a 1 (leve) ou 2 (acentuado). CASI-S (Cognitive Abilities Screening Instrument -Short Form) foi usado para avaliar o registro, orientação temporal, fluência verbal e evocação. Um teste de cópia de pentágonos foi acrescentado. RESULTADOS: Os RP mais frequentes nos pacientes e controles foram o de sucção (77% e 62%, respectivamente) e "snout" (60% e 27%), seguidos do glabelar (30% e 19%), paratonia (37% e 5%) e palmomentoniano (23% e 5%). Nenhum sujeito controle teve mais que três RP. A frequência dos RP tendeu a aumentar com a idade e a deterioração cognitiva. O reflexo de preensão e o sinal de Babinski foram encontrados apenas nos pacientes com DA. Os RP não se correlacionaram uns com os outros, exceto o reflexo "snout" com o de sucção e com o glabelar. CONCLUSÃO: O achado de reflexo de preensão e sinal de Babinski, ou a presença de mais de três sinais primitivos, particularmente a combinação de paratonia e reflexos "snout", sucção e palmomentoniano, são sugestivos de disfunção cerebral, especialmente quando esses sinais são acentuados e acompanhados de déficits de orientação, evocação, fluência verbal e praxia construcional

P-29 CLINICAL FEATURES OF PRIMARY BILIARY CHOLANGITIS IN BRAZIL

Little is known about primary biliary cholangitis (PBC) in Latin America, where this disease is thought to be rare. To analyze clinical and biochemical features of Brazilian PBC patients. The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical and laboratory features from PBC patients. 562 patients with PBC were included; 80 (14.2%) had overlapping syndrome with autoimmune hepatitis and were excluded. Most subjects were middle-aged women (95%; mean age 51 ± 11 years) with classical symptoms of pruritus and/or fatigue (65%) and jaundice (22%). Mean time to diagnosis was 2.5 years. Prevalence of antimitochondrial (AMA) and antinuclear antibodies was 82.8% and 72.1%, respectively. Concurrent autoimmune diseases occurred in 18.9%, mainly Hashimoto's thyroiditis and Sjogren syndrome. Celiac disease was diagnosed in 1:80 (1.2%). Osteopenia and osteoporosis were demonstrated in 42% and 26%, respectively. Liver pathology at diagnosis was available for 326 patients (67.6%). One third of them had advanced PBC. After a mean follow-up of 6.2 ± 5.3 years, 32% of the subjects had clinical, laboratory or imaging evidence of cirrhosis. Requirement for liver transplantation and liver-related deaths were reported in 6.6% and 3.2% of the patients, respectively. Hepatocarcinoma was diagnosed in 1.9% of the subjects. A higher predominance of PBC among females, compared to other populations, was observed, while AMA positivity was lower. Concurrent autoimmune, celiac and bone diseases are common and should be adequately screened. Prolonged time to diagnosis and high prevalence of advanced liver disease might reflect difficulties in health care access in Brazil