ABSTRACT
Objective: To explore the clinical and genetic characteristics of children with dopa-responsive dystonia (DRD) caused by tyrosine hydroxylase (TH) gene variations. Methods: Clinical data of 9 children with DRD caused by TH gene variations diagnosed in the Department of Children Rehabilitation, the Third Affiliated Hospital of Zhengzhou University from January 2017 to August 2022 were retrospectively collected and analyzed, including the general conditions, clinical manifestations, laboratory tests, gene variations and follow-up data. Results: Of the 9 children with DRD caused by TH gene variations, 3 were males and 6 were females. The age at diagnosis was 12.0 (8.0, 15.0) months. The initial symptoms of the 8 severe patients were motor delay or degression. Clinical symptoms of the severe patients included motor delay (8 cases), truncal hypotonia (8 cases), limb muscle hypotonia (7 cases), hypokinesia (6 cases), decreased facial expression (4 cases), tremor (3 cases), limb dystonia (3 cases), diurnal fluctuation (2 cases), ptosis (2 cases), limb muscle hypertonia (1 case) and drooling (1 case). The initial symptom of the very severe patient was motor delay. Clinical symptoms of the very severe patient included motor delay, truncal hypotonia, oculogyric crises, status dystonicus, hypokinesia, decreased facial expression, and decreased sleep. Eleven TH gene variants were found, including 5 missense variants, 3 splice site variants, 2 nonsense variants, and 1 insertion variant, as well as 2 novel variants (c.941C>A (p.T314K), c.316_317insCGT (p.F106delinsSF)). Nine patients were followed up for 40 (29, 43) months, and no one was lost to follow-up. Seven of the 8 severe patients were treated by levodopa and benserazide hydrochloride tablets and 1 severe patient was treated by levodopa tablets. All the severe patients responded well to levodopa and benserazide hydrochloride tablets or levodopa tablets. Although the weight of the patients increased and the drug dosage was not increased, the curative effect remained stable and there was no obvious adverse reaction. One severe patient developed dyskinesia in the early stage of treatment with levodopa and benserazide hydrochloride tablets and it disappeared after oral administration of benzhexol hydrochloride tablets. Until the last follow-up, motor development of 7 severe patients returned to normal and 1 severe patient still had motor delay due to receiving levodopa and benserazide hydrochloride tablets for only 2 months. The very severe patient was extremely sensitive to levodopa and benserazide hydrochloride tablets and no improvement was observed in this patient. Conclusions: Most of the DRD caused by TH gene variations are severe form. The clinical manifestations are varied and easily misdiagnosed. Patients of the severe patients responded well to levodopa and benserazide hydrochloride tablets or levodopa tablets, and it takes a long time before full effects of treatment become established. Long-term effect is stable without increasing the drug dosage, and no obvious side effect is observed.
Subject(s)
Female , Humans , Infant , Male , Benserazide/therapeutic use , Dystonia/genetics , Hypokinesia/drug therapy , Levodopa/pharmacology , Muscle Hypotonia , Retrospective Studies , Tyrosine 3-Monooxygenase/geneticsSubject(s)
Humans , Female , Adult , Antiphospholipid Syndrome/diagnostic imaging , Hypokinesia/diagnostic imaging , Acute Coronary Syndrome/therapy , Acute Coronary Syndrome/diagnostic imaging , Anomalous Left Coronary Artery/pathology , Chest Pain , Echocardiography/methods , Cardiac Catheterization/methods , Heparin/chemistry , Enoxaparin/chemistry , Electrocardiography/methods , Computed Tomography Angiography/methods , Anticoagulants/chemistryABSTRACT
Objective: To evaluate of the microstructure of the salivary glands of experimental animals born from of females rats, who were subjected to different modes of motor activity during the pregnancy: limited and compensated by regular moderate training. Material and Methods: The sample was composed 84 three-month-old descendants of Wistar rats which were divided into three groups: G1 (n = 27): posterity of female rats, who during gestation were in standard conditions; G2 (n = 25): posterity of female rats, who during pregnancy were in hypokinesia conditions; G3 (n = 32): which stayed in conditions of hypokinesia, but had running load in running wheel regularly 5 times a week. Results: In adult descendants of G2, morphological structure of the submandibular salivary glands was characterized by decrease size and number of glandular cells; reduction of their cytoplasm; increase in heterochromatin amount and decrease in the intensity of fuchsinophilia. Microstructure of the submandibular salivary glands of the offspring of G3 indicates positive effect of regular short-term physical activity on the morphofunctional state of the specified glands. An increased number of tissue basophils in their stroma is also in favor of greater functional activity of salivary glands. Conclusion: This study indicates deceleration of the metabolic processes in salivary glands of animals under the influence of hypokinesia of their mothers during pregnancy. It was also confirmed that regular physical activity completely eliminates the negative effect of mothers' hypokinesia on morphology the salivary glands of the descendant.
Subject(s)
Animals , Pregnancy , Rats , Salivary Glands , Submandibular Gland , Morphological and Microscopic Findings , Morphogenesis , Motor Activity , Ukraine , Pregnancy , Rats, Wistar , Hypokinesia , Animal ExperimentationABSTRACT
Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder of an unknown origin. The role of leptospirosis as a triggering factor for SLE is unknown. This paper reports an uncommon case of SLE following a leptospira infection. A 29-year-old female was referred due to fevers, myalgia, and facial edema with rash. Laboratory investigations revealed a hepatic dysfunction, significantly raised lactate dehydrogenase with marked leukopenia and thrombocytopenia. A diagnosis of leptospirosis was confirmed. The patient was treated with antibiotic therapy for leptospirosis. She developed dyspnea after one week. The echocardiogram revealed global hypokinesia with a decreased ejection fraction. A positivity of antinuclear, anti-DNA, and anti-Smith antibodies, together with clinical and laboratory improvement by steroid therapy, led to the diagnosis of SLE. This case highlights the presence of concurrent SLE and leptospirosis. As the symptoms of SLE are similar to leptospirosis, accurate diagnosis through high suspicion is essential for appropriate treatment.
Subject(s)
Adult , Female , Humans , Antibodies , Diagnosis , Dyspnea , Edema , Exanthema , Fever , Hypokinesia , L-Lactate Dehydrogenase , Leptospira , Leptospirosis , Leukopenia , Lupus Erythematosus, Systemic , Myalgia , Myocarditis , ThrombocytopeniaABSTRACT
BACKGROUND: Parkinson's disease (PD) is one of the most common neurodegenerative diseases. However, the history of PD and famous persons with PD have not been described in detail yet. METHODS: We summarized the history of PD before the first description of James Parkinson's. The four famous patients who were suspected or diagnosed with PD were reviewed through peer-reviewed journals as well as biographies, books, and media. RESULTS: Before the definition of PD was established, there were descriptions of various Parkinsonian symptoms in several literatures. The diagnoses of Adolf Hitler and Na Hyeseok are not certain and we only suspect that they had parkinsonism. The diagnoses of PD of the Pope John Paul II and Muhammad Ali are certain as they had medical records as well as video records that shows progressive deterioration. CONCLUSIONS: Even before James Parkinson, PD have been recognized and described focusing on the bradykinesia and tremor. We should keep in mind that detailed examination as well as transcriptions are important, and that long-term follow-up is needed to document or differentiate PD and its mimics.
Subject(s)
Humans , Diagnosis , Famous Persons , Follow-Up Studies , Hypokinesia , Medical Records , Neurodegenerative Diseases , Parkinson Disease , Parkinsonian Disorders , TremorABSTRACT
RESUMO Objetivo Verificar o efeito imediato e após 15 minutos da técnica de sobrearticulação de fala em indivíduos com doença de Parkinson, submetidos à estimulação cerebral profunda em núcleo subtalâmico, nos parâmetros de voz, fala e mímica facial. Métodos Participaram 29 sujeitos com diagnóstico médico de doença de Parkinson, sob tratamento medicamentoso e submetidos à estimulação cerebral profunda em núcleo subtalâmico, com e sem reabilitação fonoaudiológica prévia. Realizou-se gravação em áudio e vídeo de amostra de fala e voz em três momentos: pré-intervenção, pós-imediato e após 15 minutos da realização individual de cinco minutos de uma sequência de exercícios de sobrearticulação de fala. As gravações foram submetidas à avaliação perceptivo-auditiva da voz e perceptivo-visual da mímica facial. Resultados A técnica produziu resultados positivos em 69% dos casos, após 15 minutos de aplicação, em relação aos demais momentos. O parâmetro que mais contribuiu para a identificação da melhor emissão na avaliação perceptivo-auditiva foi articulação (69%), significativamente maior que os demais parâmetros, exceto velocidade de fala. A análise perceptivo-visual mostrou melhora em 58,6% dos casos após 15 minutos, também significativamente melhor que os demais momentos. O parâmetro em que se observou maior proporção de melhora foi na movimentação de boca, sobrancelhas e olhos. Conclusão A técnica de sobrearticulação de fala produz efeito positivo imediato nos aspectos vocais e uma maior expressividade facial, principalmente após 15 minutos de realização.
ABSTRACT Purpose To verify the immediate and after 15 minutes effect of the overarticulation technique in individuals with Parkinson's disease, submitted to deep brain stimulation in subthalamic nucleus, in the voice, speech and facial movements. Methods This study counted with 29 patients with the diagnosis of Parkinson Disease who were undergoing pharmacotherapy and were submitted to deep brain stimulation in the subthalamic nucleus, with and without prior speech therapy. Speech and voice samples were recorded in an audio and video file at three different moments: pre-intervention, immediate post intervention and 15 minutes post intervention. The intervention was the individual performance of 5 minutes exercise consisting of sequence of overarticulation techniques. The audio and video recordings were submitted to the perceptual-judgement of the voice quality and facial movements. Results The technique produced positive results in 69% of the cases after 15 minutes of its application when compared to the other moments. Articulation was the parameter that most contributed in the perceptual-judgment of the best voice quality (69%); it was significantly higher than the other parameters, except speech rate. After 15 minutes of the technique, 58.6% of the patients had improvement in the facial movements, according to the perceptual-judgment which was also significantly better when compared to other moments. The parameter with greater provement was movement of the mouth, eyebrows and eyes. Conclusion The overarticulation technique produces an immediate positive effect on vocal aspects and greater facial expressiveness, especially after 15 minutes.
Subject(s)
Humans , Parkinson Disease/therapy , Speech Therapy/methods , Voice Disorders/rehabilitation , Deep Brain Stimulation , Dysarthria/rehabilitation , Parkinson Disease/surgery , Prospective Studies , Hypokinesia/rehabilitation , Subthalamic Nucleus/surgery , Facial ExpressionABSTRACT
The current body of literature contains 5 reports of myotonic dystrophy (DM) with parkinsonism: 4 reports of DM type 2 and 1 report of clinically suspected DM type 1. To date, there have been no genetically proven cases of DM type 1 with parkinsonism. Here, we report the first case of genetically proven DM type 1 and parkinsonism that developed ahead of muscle symptoms with bilateral putaminal, presynaptic dopaminergic deficits on imaging. A 54-year-old female patient presented with bradykinesia, axial and bilateral limb rigidity, stooped posture, and hypomimia, which did not respond to levodopa. At age 56, she developed neck flexion weakness. Examination showed bilateral facial weakness, percussion and grip myotonia, and electromyography confirmed myotonic discharges. A genetic study of DM type 1 showed a DMPK mutation. At age 58, gait freezing, postural instability, and frequent falling developed and did not respond to increasing doses of levodopa. At age 59, the patient died from asphyxia.
Subject(s)
Female , Humans , Middle Aged , Accidental Falls , Asphyxia , Electromyography , Extremities , Freezing , Gait , Hand Strength , Hypokinesia , Levodopa , Myotonia , Myotonic Dystrophy , Neck , Parkinsonian Disorders , Percussion , PostureABSTRACT
ABSTRACT The Bereitschaftspotential (BP) is a negative wave observed in EEG retrograde averaging, preceding a motor act. The objective was to study the BP preceding voluntary eyelid blinks in Parkinson's disease (PD) patients during off and on phases of levodopa. Methods Ten PD patients in stages 1 and 2 of the Hoehn & Yahr classification were compared to 18 healthy controls. Artifact-free EEG segments of two seconds preceding the onset of the blink potential were averaged and analyzed, and the statistical significance of the measured amplitudes were evaluated by analysis of variance models. Results The presence of a BP in the PD patients was demonstrated. The mean amplitudes at 0 ms were respectively 0.6 µV and 3.3 µV for the BP patients and the normal controls, respectively. Conclusions The BP amplitudes were significantly smaller in PD patients than normal participants. The amplitudes of the BP were not modified by levodopa.
RESUMO O Potencial de Bereitschafts (PB) é uma onda negativa observada retrogradamente no EEG precedendo um ato motor. Objetivo Estudar o PB precedendo o piscamento palpebral voluntário em pacientes com doença de Parkinson (DP) durante as fases off e on da levodopa. Foram comparados dez pacientes com DP nos estágios 1 e 2 de Hoehn & Yahr com 18 controles saudáveis. Os segmentos de EEG livres de artefatos 2 segundos antes do início do potencial foram calculados e analisados e a significância estatística das amplitudes foi medida por modelos de análise de variância. Resultados A presença de PB nos pacientes com DP foi demonstrada. As amplitudes médias a 0 ms foram respectivamente 0,6 μV e 3,3 μV para os pacientes com DP e controles respectivamente. Conclusões As amplitudes do PB foram significativamente menores nos pacientes com DP do que controles. As amplitudes do PB não foram modificadas pela levodopa.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Blinking/physiology , Levodopa/adverse effects , Contingent Negative Variation/physiology , Motor Cortex/physiology , Movement/physiology , Antiparkinson Agents/adverse effects , Parkinson Disease/physiopathology , Parkinson Disease/drug therapy , Time Factors , Case-Control Studies , Analysis of Variance , Hypokinesia/etiology , Electrodes, Implanted , Electroencephalography , Eyelids/physiologyABSTRACT
OBJECTIVE: Patients with drug-induced parkinsonism (DIP) may have nigrostriatal dopaminergic degeneration. We studied the clinical features that may indicate nigrostriatal dopaminergic degeneration in patients with DIP. METHODS: Forty-one DIP patients were classified into normal and abnormal [¹⁸F] FP-CIT scan groups. Differences in 32 clinical features and drug withdrawal effects were studied. RESULTS: Twenty-eight patients had normal (Group I) and 13 patients had abnormal (Group II) scans. Eight patients of Group I, but none of Group II, had taken calcium channel blockers (p = 0.040). Three patients of Group I and six of Group II had hyposmia (p = 0.018). After drug withdrawal, Group I showed greater improvement in Unified Parkinson's Disease Rating Scale total motor scores and subscores for bradykinesia and tremors than Group II. Only hyposmia was an independent factor associated with abnormal scans, but it had suboptimal sensitivity. CONCLUSION: None of the clinical features were practical indicators of nigrostriatal dopaminergic degeneration in patients with DIP.
Subject(s)
Humans , Calcium Channel Blockers , Dopamine Plasma Membrane Transport Proteins , Hypokinesia , Parkinson Disease , Parkinsonian Disorders , Positron-Emission Tomography , TremorABSTRACT
BACKGROUND: Evaluation of acute chest pain in emergency department (ED), using limited resource and time, is still very difficult despite recent development of many diagnostic tools. In this study, we tried to determine the applicability of new semi-automated cardiac function analysis tool, velocity vector imaging (VVI), in the evaluation of the patients with acute chest pain in ED. METHODS: We prospectively enrolled 48 patients, who visited ED with acute chest pain, and store images to analyze VVI from July 2005 to July 2007. RESULTS: In 677 of 768 segments (88%), the analysis by VVI was feasible among 48 patients. Peak systolic radial velocity (V(peak)) and strain significantly decreased according to visual regional wall motion abnormality (V(peak), 3.50 ± 1.34 cm/s for normal vs. 3.46 ± 1.52 cm/s for hypokinesia, 2.51 ± 1.26 for akinesia, p < 0.01; peak systolic radial strain -31.74 ± 9.15% fornormal, -24.33 ± 6.28% for hypokinesia, -20.30 ± 7.78% for akinesia, p < 0.01). However, the velocity vectors at the time of mitral valve opening (MVO) were directed outward in the visually normal myocardium, inward velocity vectors were revealed in the visually akinetic area (V(MVO), -0.85 ± 1.65 cm/s for normal vs. 0.10 ± 1.46 cm/s for akinesia, p < 0.001). At coronary angiography, V(MVO) clearly increased in the ischemic area (V(MVO), -0.88+1.56 cm/s for normal vs. 0.70 + 2.04 cm/s for ischemic area, p < 0.01). CONCLUSION: Regional wall motion assessment using VVI showed could be used to detect significant ischemia in the patient with acute chest pain at ED.
Subject(s)
Humans , Chest Pain , Coronary Angiography , Emergencies , Emergency Service, Hospital , Hypokinesia , Ischemia , Mitral Valve , Myocardium , Prospective Studies , ThoraxABSTRACT
As principais causas de tremor em pacientes atendidos na Atenção Primária à Saúde são: exacerbação de tremor fisiológico, tremor essencial (acomete 5% da população acima de 40 anos) e as síndromes parkinsonianas. É importante definir corretamente sua origem, pois o tratamento e o prognóstico são variados. Esta guia apresenta informação que orienta a conduta para casos de tremor e síndromes parkinsonianas no contexto da Atenção Primária à Saúde, incluindo: etiologia do tremor e síndromes parkinsonianas, avaliação clínica, tipos de tremor, sintomas cardinais de parkinsonismo, medicamentos indutores, fluxograma de avaliação do tremor, exames complementares, tratamento do tremor essencial e doença de parkinson, encaminhamento para serviço especializado.
Subject(s)
Humans , Parkinsonian Disorders/diagnosis , Parkinsonian Disorders/therapy , Essential Tremor/diagnosis , Essential Tremor/therapy , Primary Health Care , Referral and Consultation , Antipsychotic Agents/therapeutic use , Calcium Channel Blockers/therapeutic use , Hypokinesia , Antiemetics/therapeutic useABSTRACT
ABSTRACT Objective Patients with Parkinson’s disease (PD) may present with unusual motor and non-motor symptoms and signs in the early stage of the disease. Methods Cases were collected over a five-year period at two tertiary movement disorders clinics. All had a diagnosis of PD with unusual presentations defined retrospectively as the presence of complaints not objectively related to any of the classic cardinal signs of parkinsonism or the typical early non-motor features of PD. Results A total of 15 early PD patients fulfilled the proposed criteria, presenting with symptoms such as atypical tremors, shoulder pain, signs related to the rigid akinetic syndrome, as well as cases of asthenia, rhinorrhea, parosmia, dysgeusia, nocturnal sialorrhea, and color discrimination disorders. Conclusions Unusual motor and non-motor symptoms and signs in the early stage of PD can be difficult to interpret. Specialists should be aware of these conditions as clues to a potential diagnosis.
RESUMO Objetivo Pacientes com doença de Parkinson (DP) podem apresentar sintomas e sinais motores e não motores pouco comuns na fase inicial da doença. Métodos Os casos foram coletados em um período de cinco anos, em dois centros terciários de distúrbios do Movimento. Todos os pacientes tinham o diagnóstico de DP com apresentações clínicas iniciais pouco comuns. Resultados Um total de 15 pacientes com DP na fase inicial, apresentando sintomas e sinais tais como, tremores atípicos, dor no ombro, sinais relacionados com a síndrome rígido-acinética, bem como casos com astenia, rinorréia, parosmia, disgeusia, sialorréia noturna e distúrbios da discriminação de cores. Conclusões Sintomas e sinais motores e não motores pouco comuns na fase inicial da DP podem ser de difícil interpretação. Neurologistas devem estar a par destas condições, como pistas para o potencial diagnóstico.
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Parkinson Disease/diagnosis , Parkinson Disease/physiopathology , Symptom Assessment , Motor Disorders/diagnosis , Motor Disorders/physiopathology , Tremor/diagnosis , Tremor/physiopathology , Brazil , Retrospective Studies , Hypokinesia/diagnosis , Hypokinesia/physiopathology , Early Diagnosis , Motor Activity/physiologyABSTRACT
PURPOSE: To investigate the role of adenosine A2A receptors on 6-OHDA-induced motor disorder in rat. METHODS: In order to induce experimental model of Parkinson's disease, 6-hydoxydopamine (8 μg/rat) was injected unilaterally into the SNc. After three weeks as a recovery period, 6-OHDA-induced bradykinesia and balance disturbances were assessed by using beam traversal test 10, 30 and 60 minutes after intraperitoneal injections of the drugs (caffeine, SCH58261). RESULTS: The results showed that 6-OHDA (8 μg/rat, Intra-SNc) induced motor disorders of Parkinson's disease and increased elapsed time in the beam test (p<0.001). Injection of caffeine (30 mg/kg, i.p.) and SCH58261 (2 mg/kg, i.p.) attenuated elapsed time on beam (p<0.01 and p<0.001). We showed that acute administration of caffeine and SCH 58261 can improve the 6-OHDA-induced bradykinesia and motor disturbance. CONCLUSION: Adenosine A2AR antagonists improve 6-OHDA-motor deficit and this effect seems to be mediated by the inhibition of A2A presynaptic receptors in substantia nigra pars compacta.
Subject(s)
Animals , Male , Parkinson Disease, Secondary/chemically induced , Caffeine/pharmacology , Oxidopamine/adverse effects , Purinergic P1 Receptor Antagonists/pharmacology , Adenosine A2 Receptor Antagonists/pharmacology , Time and Motion Studies , Rats, Wistar , Hypokinesia/chemically induced , Disease Models, Animal , Motor Disorders/chemically induced , Motor Activity/drug effectsABSTRACT
BACKGROUND AND PURPOSE: Our aims were to analyze the characteristics of parkinsonian features and to characterize changes in parkinsonian motor symptoms before and after the cerebrospinal fluid tap test (CSFTT) in idiopathic normal-pressure hydrocephalus (INPH) patients. METHODS: INPH subjects were selected in consecutive order from a prospectively enrolled INPH registry. Fifty-five INPH patients (37 males) having a positive response to the CSFTT constituted the final sample for analysis. The mean age was 73.7±4.7 years. The pre-tap mean Unified Parkinson's Disease Rating Scale motor (UPDRS-III) score was 24.5±10.2. RESULTS: There was no significant difference between the upper and lower body UPDRS-III scores (p=0.174). The parkinsonian signs were asymmetrical in 32 of 55 patients (58.2%). At baseline, the Timed Up and Go Test and 10-meter walking test scores were positively correlated with the total motor score, global bradykinesia score, global rigidity score, upper body score, lower body score, and postural instability/gait difficulties score of UPDRS-III. After the CSFTT, the total motor score, global bradykinesia score, upper body score, and lower body score of UPDRS-III significantly improved (p<0.01). There was a significant decrease in the number of patients with asymmetric parkinsonism (p<0.05). CONCLUSIONS: In the differential diagnosis of elderly patients presenting with asymmetric and upper body parkinsonism, we need to consider a diagnosis of INPH. The association between gait function and parkinsonism severity suggests the involvement of similar circuits producing gait and parkinsonian symptoms in INPH.
Subject(s)
Aged , Humans , Cerebrospinal Fluid , Diagnosis , Diagnosis, Differential , Gait , Hydrocephalus , Hydrocephalus, Normal Pressure , Hypokinesia , Parkinson Disease , Parkinsonian Disorders , Prospective Studies , WalkingABSTRACT
OBJECTIVE: The aim of this subgroup analysis was to identify the risk factors associated with the development of various movement disorder phenotypes. METHODS: Eighty-three non-Wilsonian cirrhotic patients with abnormal movements were allocated into the following groups: intention tremor, bradykinesia, Parkinsonism, and abnormal ocular movements. These movement types were considered the primary outcomes as there was a sufficient sample size. Researchers took into consideration the gender, etiologies of cirrhosis, cirrhosis-related complications, hepatic encephalopathy, medical illness, and some neurological deficits as potential factors associated with these movement disorders. RESULTS: The male gender (p = 0.002) and alcoholic cirrhosis (p = 0.005) were significant factors for the prevalence of intention tremors. In bradykinesia, hepatic encephalopathy was highly statistically significant (p < 0.001), and females more commonly developed bradykinesia (p = 0.04). The Parkinsonism features in this study were confounded by hyperlipidemia (p = 0.04) and motor or sensory deficits (p = 0.02). Jerky pursuits and a horizontal nystagmus were detected. Jerky pursuits were significantly related to hepatic encephalopathy (p = 0.003) and bradykinesia, but there were no factors associated with the prevalence of nystagmus other than an intention tremor. CONCLUSIONS: The association of alcoholic cirrhosis with the development of intention tremor indicates that the persistent cerebellar malfunction in cirrhotic patients is due to alcohol toxicity. The slowness of finger tapping and jerky pursuit eye movements are significantly associated with hepatic encephalopathy. Thus, further studies are needed to evaluate the diagnostic value of these two signs for an early detection of mild hepatic encephalopathy.
Subject(s)
Female , Humans , Male , Dyskinesias , Eye Movements , Fibrosis , Fingers , Hepatic Encephalopathy , Hyperlipidemias , Hypokinesia , Liver Cirrhosis, Alcoholic , Movement Disorders , Nystagmus, Pathologic , Parkinsonian Disorders , Phenotype , Prevalence , Risk Factors , Sample Size , TremorABSTRACT
A 77-year-old woman was scheduled for a coronary artery bypass graft. Her preoperative transthoracic echocardiographic (TTE) examination revealed an enlarged left atrium with reduced systolic dysfunction (ejection fraction: 38%), moderate global hypokinesia of the left ventricle, and moderate mitral and tricuspid regurgitation. No thrombus was visualized on the preoperative TTE. However, the intraoperative transesophageal echocardiography performed before the cardiopulmonary bypass revealed a thrombus of approximately 1.3 × 1.8 cm in the left atrial appendage (LAA). The LAA thrombus was removed, an internal suture was placed on the LAA before the coronary artery bypass grafting, and the main operation was performed successfully. The patient was transferred to the intensive care unit to receive postoperative care. She was extubated 4 h after the surgery and was transferred to the general ward on postoperative day 3 without any neurological sequelae.
Subject(s)
Aged , Female , Humans , Atrial Appendage , Cardiopulmonary Bypass , Coronary Artery Bypass , Coronary Vessels , Echocardiography , Echocardiography, Transesophageal , Heart Atria , Heart Ventricles , Hypokinesia , Intensive Care Units , Patients' Rooms , Postoperative Care , Sutures , Thrombosis , Transplants , Tricuspid Valve InsufficiencyABSTRACT
OBJECTIVE: Parkinsonism and other movement disorders have previously been reported in the acquired hepatocerebral degeneration associated with portosystemic shunting. However, there is no study to date about their prevalence as has been noted in general practice. METHODS: One hundred and forty-three patients with hepatic cirrhosis from the gastroenterology clinic and internal medicine wards were enrolled. Liver data included the diagnoses, etiologies, assessments of complications, and treatments for cirrhosis. Hepatic encephalopathy was classified with regard to the West Haven criteria for semi-quantitative grading for mental status. Neurological examination results and abnormal involuntary movements were recorded as primary outcomes. Neuro-radiology was used for the detection of severe brain lesions. RESULTS: Alcoholism was the most common cause of liver cirrhosis. Eighty-three patients (58%) presented with movement disorders. Asterixis was found in one of the cases. The most common movement disorder seen was an intentional tremor at 37.1%, which was followed by bradykinesia, Parkinsonism, and postural tremors at 29.4%, 10.5%, and 6.3%, respectively. The prevalence of movement disorders simultaneously increased with a high Child-Turcotte-Pugh score. The hepatic encephalopathy was grade 1 and 2. With the inclusion of age-range adjustments, we found that alcoholic cirrhosis and hepatic encephalopathy are statistically significant factors [p < 0.05, odds ratio (OR) = 6.41, 95% confidence interval (CI) 1.38-29.71 and p < 0.001, OR = 13.65, 95% CI 4.71-39.54] for the development of movement disorders in non-Wilsonian cirrhotic patients. CONCLUSIONS: Intentional tremor is a common abnormal movement. Alcoholic cirrhosis and hepatic encephalopathy are significant risk factors in the development of movement disorders in non-Wilsonian cirrhotic patients.
Subject(s)
Humans , Alcoholism , Brain , Diagnosis , Dyskinesias , Fibrosis , Gastroenterology , General Practice , Hepatic Encephalopathy , Hepatolenticular Degeneration , Hypokinesia , Internal Medicine , Liver , Liver Cirrhosis , Liver Cirrhosis, Alcoholic , Movement Disorders , Neurologic Examination , Odds Ratio , Parkinsonian Disorders , Portasystemic Shunt, Surgical , Prevalence , Risk Factors , Schools, Medical , TremorABSTRACT
A 70-year-old woman underwent cardiopulmonary bypass surgery for aorta dissection. After 10 days she developed a vertical gaze palsy, and 2 months later she presented with dysarthria, bradykinesia, postural instability, blepharospasm, and truncal tilt to the left. Brain imaging indicated old lacunes in the bilateral thalamus. Her symptoms remained unchanged during a 4-year follow-up, which seems to be incompatible with progressive supranuclear palsy (PSP). However, the clinical features of this case were suggestive of PSP-like syndrome after cardiopulmonary bypass surgery.
Subject(s)
Aged , Female , Humans , Aorta , Blepharospasm , Cardiopulmonary Bypass , Dysarthria , Follow-Up Studies , Hypokinesia , Neuroimaging , Paralysis , Parkinsonian Disorders , Supranuclear Palsy, Progressive , ThalamusABSTRACT
A 65-year-old female visited us due to gait disturbance. A neurological examination showed cognitive impairment, dystonia, myoclonus, bradykinesia, postural instability, and freezing of gait (FOG). She was diagnosed with extrapontine myelinolysis based on her history of hyponatremia and high signal intensities (HSIs) in both striata on T2-weighted images. Her neurological problems including FOG improved over 25 days. In a follow-up MRI 50 days after the onset, HSIs disappeared in the striata but new ones appeared in the pons. FOG may have been related to striatal dysfunction in this patient.