ABSTRACT
Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.
A paralisia supranuclear progressiva (PSP) é uma doença neurodegenerativa, que afeta principalmente o tronco cerebral e os núcleos da base. O quadro clínico se caracteriza por oftalmoparesia supranuclear, instabilidade postural e demência . Do ponto de vista anátomo-patológico, a PSP se caracteriza por acúmulo de emaranhados neurofibrilares no núcleo subtalâmico, globo pálido, núcleo rubro, substância negra, estriado, tegumento da ponte, núcleos oculomotores, bulbo e núcleo denteado. Nas últimas décadas, muitas linhas de pesquisa têm colaborado para redefinir a PSP em muitos aspectos. Os objetivos dessa revisão são auxiliar o neurologista geral na identificação da doença, compreensão da sua fisiopatologia, além de apresentar alternativas para seu tratamento sintomático.
Subject(s)
Humans , Supranuclear Palsy, Progressive , Supranuclear Palsy, Progressive/classification , Supranuclear Palsy, Progressive/diagnosis , Supranuclear Palsy, Progressive/physiopathology , Supranuclear Palsy, Progressive/therapyABSTRACT
Two cases of progressive anarthria are reported; we remark their close but distinct relation with speech apraxia. Both of them were older female, with a progressive loss of speech, bilateral paresis of lower face, tongue and palatal muscles. They also had mild pyramidal signs and a fronto-subcortical cognitive deterioration. Brain TC and MRI were within normal limits. One of them had a possible progressive supranuclear palsy, the other one a possible corticobasal degeneration. The analysis of similar cases reports let us to conclude than there are several pathologies that can cause a progressive pseudobulbar palsy. The final diagnosis must be by postmortem examination of the brain.
Se presentan dos casos de anartria progresiva, discutiendo la relación o el diagnóstico diferencial con la apraxia del habla progresiva. En ambos casos se trataba de mujeres mayores de 65 años con un cuadro de pérdida progresiva del lenguaje oral, con diparesia facial, lingual y velar, deterioro cognitivo de tipo frontal y discretos signos piramidales. Las imágenes cerebrales estructurales fueron normales. Uno de ellos pudo corresponder a una parálisis supranuclear progresiva, la otra a una degeneración corticobasal. Se analiza la literatura, llegando a la conclusión de que existen una serie de cuadros que pueden presentarse con un síndrome pseudobulbar progresivo. El diagnóstico definitivo debiera ser patológico.
Subject(s)
Humans , Female , Aged , Apraxias/diagnosis , Apraxias/physiopathology , Dysarthria/diagnosis , Dysarthria/physiopathology , Cerebral Cortex/physiopathology , Diagnosis, Differential , Neurodegenerative Diseases/physiopathology , Basal Ganglia/physiopathology , Pseudobulbar Palsy , Supranuclear Palsy, Progressive/physiopathology , Disease Progression , Speech Disorders/etiologyABSTRACT
BACKGROUND: Few studies have compared cognitive functions in multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and Parkinson's disease (PD). AIM: To compare the results of cognitive function tests in the three diseases and examine their relation with the severity of parkinsonism. SETTINGS AND DESIGN: Clinic-based open prospective study. MATERIALS AND METHODS: Global cognitive function tests and tests specific for frontal lobe functions were used in 25 cases of each disease. UPDRS III was used to measure the severity of parkinsonism. STATISTICAL ANALYSIS: ANOVA was done for group comparisons, followed by t-test for independent samples with Bonferroni correction. Pearson's correlation test was done to assess the relation between severity of parkinsonism and cognitive functions. RESULTS: The severity of parkinsonism was worst in PD followed by PSP and least in MSA. Patients with PSP exhibited the worst performance in both sets of cognitive tests. Even though patients with MSA did better than PD in global function tests, they performed worse than PD in some frontal function tests. There was a negative correlation between severity of parkinsonism and scores in cognitive tests in the MSA group but not in others. CONCLUSIONS: Global and frontal dysfunction was worst in PSP. The frontal dysfunction in MSA was more severe than PD, correlated with the severity of parkinsonism and was worse in clinically probable than possible cases of MSA. The severity of cognitive dysfunction in these diseases may be related to the distribution and extent of pathological changes affecting the striato-frontal circuits in them.
Subject(s)
Aged , Analysis of Variance , Cognition/physiology , Demography , Female , Humans , Male , Middle Aged , Multiple System Atrophy/physiopathology , Neuropsychological Tests/statistics & numerical data , Parkinson Disease/physiopathology , Retrospective Studies , Supranuclear Palsy, Progressive/physiopathologyABSTRACT
La parálisis supranuclear progresiva (PSP), también llamada síndrome de Steele-Richardson-Olszewski, es uno de los síndromes parkinsionanos atípicos más frecuentes. Es una afección degenerativa de causa desconocida caracterizada por atrofia del tegmentum protuberancial y mesencéfalo, decoloración de la sustancia nigra y relativo respeto de la corteza cerebral. Existe un consenso sobre la dificultad para llegar al diagnóstico de certeza tanto clínico como patológico. Ha habido casos de PSP clínica cuyas autopsias no confirmaron el diagnóstico, y autopsias con hallazgos diagnósticos de PSP que fueron interpretados clínicamente como otros padecimientos o síndromes degenerativos. En Latinoamérica no existen estadísticas epidemiológicas sobre esta entidad nosológica y en Chile no se ha publicado ningún caso hasta la fecha. El objetivo del presente reporte es la presentación de seis casos que cumplen con los criterios diagnósticos establecidos por el National Institute of Neurological Disorders and Stroke and The Society for PSP (NINDS-SPSP) en 1996. Se concluye, a partir del presente trabajo que la PSP no es excepcional en nuestro medio y que debe considerarse entre los diagnósticos diferenciales de los síndromes parkinsonianos
Subject(s)
Humans , Aged , Male , Female , Supranuclear Palsy, Progressive/diagnosis , Parkinsonian Disorders , Clinical Diagnosis , Diagnosis, Differential , Dystonia , Supranuclear Palsy, Progressive/physiopathology , Tegmentum Mesencephali/physiopathologyABSTRACT
Paralisia supranuclear progressiva (PSP) foi inicialmente identificada como uma entidade distinta por Richardson, Steele e Olszewski, há cerca de 25 anos atrás. Observaçoes clínicas subseqüentes nao apenas tem confirmado, como acrescentaram novas informaçoes aos relatos pioneiros. PSP pode ser descrita como um transtorno neurológico progressivo, com rigidez extrapiramidal, bradicinesia, dificuldade na marcha, paralisia bulbar, demência e uma oftalmoplegia supranuclear característica. Incide em indivíduos de meia idade ou idosos, sem distinçao racial ou sexual. PSP é uma importante causa de parkinsonismo e sua etiologia permanece obscura. Esta comunicaçao irá apresentar um paciente de Santa Catarina, exibindo inequívoca evidência clínica desta síndrome. Este é o primeiro caso descrito neste estado onde, baseado em recentes premissas epidemiológicas poderemos supor a existência de pelo menos mais 50 pacientes com esta condiçcao. Em complementaçcao, uma revisao da literatura com ênfase nos seus aspectos clínicos e terapêuticos é oferecida.