Biomedical subjects
Publications and source records attributed to Francisco Cardoso.
The authors analyse the correspondence between Eça de Queiroz, a well-known Portuguese writer, and his wife, in 1900, describing the neurological disease of their second son, José Maria d'Eça de Queiroz, know as Zezé. In those letters, the writer mentions chorea as his son diagnosis, explaining in detail its clinical picture. In addition, the writer reveals the medical thoughts of the time about the aetiology, treatment and natural history of Sydenham's Chorea.
Sydenham's chorea (SC) is characterized by motor, mainly choreic involuntary movements, and psychiatric symptoms, including anxiety, depression, obsessive-compulsive and attention-deficit/hyperactivity disorders. Symptoms of SC may be considered as the result of basal ganglia dysfunction determined by autoimmune mechanisms elicited by streptococcal infection. We report on a case of a 13-year-old boy with brief psychotic episode following acute SC. His hallucinations and delusions remitted after treatment with risperidone, paralleling motor improvement. The neurobiological basis of psychotic symptoms remains largely elusive. This case report provides further insight about the involvement of basal ganglia dysfunction on psychosis.
BACKGROUND: Sydenham's chorea (SC) is a disorder associated with rheumatic fever and related to Streptococcus-induced immune reaction cross-reactive with basal ganglia antigens. Obsessive-compulsive disorder and other behavioral abnormalities have been described in SC. There are scarce data of cognitive function in this condition. OBJECTIVE: To assess verbal fluency in patients with SC. PATIENTS AND METHODS: We have compared the semantic (SVF) and phonemic verbal fluency (PVF) of 20 subjects with SC and 40 controls matched by age, gender and years of school. RESULTS: The scores of the control group on the SVF and PVF tests were 26.3 +/- 5.7 and 18.0 +/- 6.4 whereas the SC patients scored 25.1 +/- 6.9 and 12.5 +/- 8.2. PVF was significantly decreased in the SC group (p < 0.01). CONCLUSION: SC is associated with reduction of PVF. This finding may reflect disruption of the dorsolateral prefrontal-striatal circuit caused by the auto-immune process characteristic of SC.
We evaluated changes in different domains of quality of life (QL) for persons with Parkinson's disease after a program of physical activity. Twenty subjects with a diagnosis of Parkinson's disease classified as Stages 1 to 3 on the Hoehn and Yahr scale and with a mean age of 61.5 +/- 9.8 years participated in 36 group sessions of a combined group program of aerobic conditioning and muscular strengthening. QL was investigated by the Nottingham Health Profile, a generic questionnaire composed of six domains. Student's paired t tests indicated significant gains associated with the program (P < 0.05) on the total score and those related to emotional reactions (ER), social interactions (SI), and physical ability (PA). SI was the domain that showed the greatest program gains (41.4%). The program of physical activity performed with persons with Parkinson's disease at light to moderate stages resulted in improvements in their perception of QL, mainly in the domains of ER, SI, and PA.
Sydenham's chorea (SC), a major manifestation of acute rheumatic fever (RF), is characterized by chorea and other motor and non-motor features. Among the latter are behavioral symptoms, including obsessive-compulsive disorder. Although SC is typically a self-limited condition, up to 50% of patients may evolve with persistent chorea. There is evidence that Gustav Mahler had a movement disorder, but its nature remains undetermined. There are witnesses describing him as having facial dyskinesia and a gait disorder consistent with chorea. His conducting performance was notorious for obsessive attention to details of the staging and musical production. Mahler was diagnosed with a valvulopathy in 1907 and died of subacute bacterial endocarditis in 1911. It is possible that the composer suffered from RF in childhood with carditis and SC, which may left him with valvulopathy, obsessive-compulsive disorder, and persistent chorea.
Currently, infectious agents are a rare cause of parkinsonism. We report on an immunocompetent patient with persistent parkinsonism associated with cryptococcal abscesses of the basal ganglia.
Several community-based surveys on the prevalence of Parkinsonism and Parkinson's disease have been conducted worldwide, with variations on their methodology and results. The objective of this study is to assess the prevalence of Parkinsonism and its causes in a cohort of individuals age 64 years or older in Bambuí, a Brazilian town. In phase I, 1,186 people older than 64 years responded to a 9-question screening questionnaire for Parkinsonism. In phase II, all subjects who scored > or = 2 points on the test were examined independently by at least 2 movement disorder-trained physicians. A movement disorder senior specialist excluded or confirmed the diagnosis in all suspected cases. The response rate was high for both phases (96% for phase I and 94% for phase II). The prevalence rate per 100 population over 64 years of age in this group was 7.2% for Parkinsonism of all types (n = 86). The most frequent causes were idiopathic Parkinson's disease and drug-induced Parkinsonism, with prevalence rates of 3.3% (n = 39) and 2.7% (n = 32), respectively. The prevalence of vascular Parkinsonism was 1.1% (n = 13). We found 1 case of posttraumatic Parkinsonism and another with multiple system atrophy. In this first population-based study of Parkinsonism conducted in Brazil, the prevalence in a cohort of elderly subjects was higher than the observed in other populations worldwide, especially because of the high rates of drug-induced and vascular Parkinsonism. The prevalence of Parkinson's disease was similar to that observed in elderly people in door-to-door surveys in other American, European, and Eastern countries.
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Chorea is one of the major types of involuntary movement disorders originating from dysfunctional neuronal networks interconnecting the basal ganglia and frontal cortical motor areas. The syndrome is characterised by a continuous flow of random, brief, involuntary muscle contractions and can result from a wide variety of causes. Diagnostic work-up can be straightforward in patients with a positive family history of Huntington's disease or acute-onset hemichorea in patients with lacunar stroke, but it can be a challenging and complex task in rare autoimmune or genetic choreas. Principles of management focus on establishing an aetiological classification and, if possible, removal of the cause. Preventive strategies may be possible in Huntington's disease where genetic counselling plays a major part. In this review we summarise the current understanding of the neuroanatomy and pathophysiology of chorea, its major aetiological classes, and principles of diagnostic work-up and management.
We report on a case of a 40 year-old man with Fahrs disease, defined by idiopathic bilateral basal ganglia calcification, who developed depressive disorder, motor and phonic tics, stereotyped behaviors such as punding and personality changes with significant social and familiar implications. We discuss about the psychopathology of Fahrs disease and the relevance of the basal ganglia in the determination of humans behavior.
Recently we developed and validated the Universidade Federal de Minas Gerais (UFMG) Sydenham's chorea Rating Scale (USCRS) to systematically assess SC patients. In this study, we assessed 97 children and adults with SC (mean age +/- SD, 15.5 +/- 5.9; male/female, 31/66) seen at the Movement Disorders Clinic at UFMG employing the USCRS. The patients were divided into 4 groups according to their clinical status: acute (n=19), recurrent (n=17), persistent (n=19) and remission (n=42). The mean +/- SEM USCRS scores for each group were: 47.7 +/- 4.7 for acute group, 29.5 +/- 2.6 for recurrent group, 17.6 +/- 3.1 for persistent group and 1.1 +/- 0.2 for remission group. All pair comparisons were statistically significant (p<0.05). Our results indicate that the USRSC can reasonably discriminate groups of SC patients in different clinical stages of the disease.
The authors investigated obsessive-compulsive behavior, obsessive-compulsive disorder (OCD), and attention deficit and hyperactivity disorder (ADHD) in 50 healthy subjects, 50 patients with rheumatic fever without chorea, and 56 patients with Sydenham chorea. Obsessive-compulsive behavior, OCD, and ADHD were more frequent in the Sydenham chorea group (19%, 23.2%, 30.4%) than in the healthy subjects (11%, 4%, 8%) and in the rheumatic fever without chorea group (14%, 6%, 8%). ADHD was more common in persistent Sydenham chorea.
Sydenham's chorea (SC) is a late complication of group A beta-hemolytic streptococci infection presumably caused by an abnormal autoimmune reaction. Despite rare case reports of peripheral neuropathy associated with streptococcal infection, there is no investigation of peripheral nerve in SC. We performed nerve conduction studies in a cohort of patients with SC. The neurophysiology investigation comprised measurement of amplitude and sensory conduction velocity of median, ulnar, and sural nerves; amplitude and motor conduction velocity; and F-wave latency of median, ulnar, fibular, and tibial nerves. Twenty-six patients entered the study (12 females, 14 males; mean age 12.8 +/- 3.6 years). Thirteen subjects had absent or decreased deep reflexes. All investigated neurophysiological parameters fell within the normal range for our population. We failed to find neurophysiological evidence of peripheral nerve involvement in patients with a history of SC. Our findings suggest that the possible autoimmune dysfunction in SC patients is not targeted against epitopes present in peripheral nerves.
Despite the renewed interest in Sydenham's chorea (SC) in recent years, there were no valid and reliable scales to rate the several signs and symptoms of patients with SC and related disorders. The Universidade Federal de Minas Gerais (UFMG) Sydenham's Chorea Rating Scale (USCRS) was designed to provide a detailed quantitative description of the performance of activities of daily living, behavioral abnormalities, and motor function of subjects with SC. The scale comprises 27 items and each one is scored from 0 (no symptom or sign) to 4 (severe disability or finding). Data from 84 subjects, aged 4.9 to 33.6 years, support the interrater reliability and internal consistency of the scale. The USCRS is a promising instrument for rating the clinical features of SC as well as their functional impact in children and adults.
The proposed pathogenesis of Sydenham's chorea (SC) is an autoantibody-mediated basal ganglia dysfunction. Our study has shown that incubation of PC12 cells with complement-inactivated serum from SC patients was associated with a significant increase in Ca2+ levels evoked by KCl stimulus (mean +/- SEM, 341.0 +/- 8.7% of fluorescence intensity, arbitrary units) when compared with incubation with control serum (313.8 +/- 8.7% of fluorescence intensity, arbitrary units; P = 0.01). The increase in Ca2+ levels determined by SC patients sera correlated directly with the enzyme-linked immunosorbent assay optical density values for anti-basal ganglia antibodies. Our study supports the hypothesis that antibodies against basal ganglia in SC may cause their dysfunction.
Serious adverse effects with antichoreic drugs can occur in patients with acute Sydenham's chorea (SC). The response to conventional treatment in severe SC may also be poor. Thus alternative therapeutic strategies have been developed. We report on four patients with severe acute SC (two with chorea paralytica and two with neuroleptic-induced parkinsonism) treated with i.v. methyl-prednisolone pulse-therapy followed by oral prednisone. The mean+/-SEM clinical rating score dropped from 63.7+/-10.2 to 33.5+/-10.6 in the first month post-treatment but in a lesser degree thereafter. Only one patient developed weight gain and 'moon facies'. This case series suggests that immune suppression with corticosteroids may be an effective and safe treatment in complicated and severe acute SC.
We report a 43-year-old woman with early-onset Parkinson's disease in whom neurological control was impaired by psychiatric co-morbidity including major depression and panic disorder. The patient also met criteria for factitious disorder that mimicked dopamine dysregulation syndrome resulting in severe clinical and social disability.
BACKGROUND: Untreated Parkinson's disease (PD) patients display speech abnormalities characterized by narrow range of fundamental frequency (Fo) variation and higher amplitude. The role of dopaminergic systems in the pathogenesis of these changes as well as the therapeutic effect of L-dopa have not been clearly determined. The aim of this study is to assess the effect of L-dopa on the prosodic features of the speech of female PD patients. METHOD: We have studied 8 PD female patients (63.5 +/- 6.8 years). The PD patients (H-Y stage 2, seven subjects; stage 2.5, four patients; stage 1, one patient) were examined when off and on. The fundamental frequency, intensity and duration of five affirmative sentences were analysed with the software WinPitch 1.8 (Philippe Martin). The analysed Fo parameters were: usual Fo (Fo us), maximal Fo (Fo max), minimal Fo (Fo min), Fo max of the prenuclear tonic (Fo max PNT), Fo min of the prenuclear tonic (Fo min PNT), Fo max of the nuclear tonic (Fo max NT), Fo min of the nuclear tonic (Fo min TN), velocity of melodic variation of the PNT (VPNT), velocity of melodic variation of the NT (VNT), amplitude of melodic variation of the PNT (APNT), and amplitude of melodic variation of the NT (ANT). Duration of the statement (D), duration of the PNT (DPNT), duration of the NT (DNT) and number of syllables per second (NSS) were the duration parameters investigated. The following intensity variables were studied: mean intensity (MI), maximal intensity (I max), minimal intensity (I min), intensity of the PNT (IPNT) and intensity of the NT (INT). Differences were statistically significant if p<0.05. RESULTS: When on, PD patients had significantly higher Fo max PNT (201.9 +/- 27.5 Hz), higher APNT (22.1 +/- 18.7 Hz), and smaller MI (32 +/- 6.2 dB) in comparison with off period (respectively: 168.5 +/- 83.5 Hz, 20.4 +/- 12.1 Hz, 32.7 +/- 5.6 dB). CONCLUSION: Our findings show that L-dopa replacement therapy slightly improves the Fo range and the intensity of speech of Brazilian female PD patients. These results suggest that non-dopaminergic systems are probably involved in the pathogenesis of prosody changes in PD. They also indicate that therapies other than dopaminergic agents are warranted to treat speech abnormalities in PD.