Progressive supranuclear palsy. Clinical and neuroimaging features

PROGRESSIVE SUPRANUCLEAR PALSY. CLINICAL AND NEUROIMAGING FEATURES (ABSTRACT)^*. DISSERTATION. SÃO PAULO, 1999.

PAULO EDUARDO MESTRINELLI CARRILHO^**

Thirty seven years ago, Steele, Richardson and Olszewski described an entity which was named progressive supranuclear palsy (PSP). Such disease is considered to be a Parkinson-plus syndrome characterized by marked postural instability, supranuclear vertical gaze abnormalities and axial rigidity. Nuchal dystonia, fronto-subcortical cognitive dysfunctions and a staring facial expression are also frequently observed. PSP is a fatal sporadic degenerative disease with a course of 6 years and it commonly appears in late sixties.

The present study is a survey of 16 probable PSP patients, diagnosed according NINDS-SPSP criteria. First clinical manifestation, presence of tremor, onset age of the disease, time to the diagnosis and L-dopa response were searched in all cases. CT-scan was performed in 16 patients, MRI in 8 and 99mTc-HMPAO¾SPECT in 9 cases. Four patients were studied with auditory (AEP), visual (VEP) and somatosensory evoked potentials (SSEP). PSP represented only 2.1% of all cases of parkinsonism in the movement disorders study group of neurology department of University of São Paulo general hospital.

The mean age of onset was 64.75 years (SD= + 7.28; min = 55 years / max = 79 years). There were a probable slight predominance of men (1.6:1).

Postural instability with falls was the most frequent initial clinical feature of PSP (62.5% of all patients). Supranuclear vertical gaze palsy was observed only 2.3 years after the disease onset. Two patients had, as a possible associated initial manifestation, ocular clinical complaints which were initially considered to be chronic dacryocystitis. Such complaints might be related with the paucity of blinking in PSP patients. Tremor was observed in 44% of all patients, although all of them developed a variable and transitory limb tremors in the beginning of the disease, only 19% presented rest tremor. Only 19% of subjects was correctly diagnosed in a first evaluation and the mean time to perform such task was 2.43 years (SD= + 1.89). L-dopa response was studied in a non-controled way and 13% of patients had a good, but transitory benefit with l-dopa.

Global atrophy was the commonest finding in CT (69%) and MRI (62,5%), suggesting that PSP was not merely a "subcortical" disease. One case, which had a definite PSP according NINDS criteria, showed putamenal low signal intensity on T2-weighted image. SPECT studies disclosed a frontal hypoperfusion in 56% of 9 patients, suggesting that PSP has a frontal dysfunction in the majority of cases. SSEP was normal in all 4 cases. AEP and VEP disclosed abnormalities in 2 patients who had the poorest performance in neuropsychological evaluation.

KEY WORDS: progressive supranuclear palsy, clinical features, neuroimage.

**Address: Rua Santa Catarina 925, 85806-000 Cascavel PR, Brasil.

*

Paralisia supranuclear progressiva: aspectos clínicos e de neuroimagem (Resumo). Dissertação de Mestrado, Faculdade de Medicina da Universidade de São Paulo (Área: Neurologia). Orientador: Ricardo Nitrini.

Publication Dates

• Publication in this collection
  05 Oct 2001
• Date of issue
  Sept 2001