| Parálisis Supranuclear
Progresiva:
Patrón de hipoperfusión de ganglios
basales y alteración frontal, en línea media, por compromiso
de las áreas premotora y motora suplementaria. Preservación
de ribete prefrontal. Demencia de desconexión.
(PSP; Huntigton; Buchpigel
(REMN vol14; nº5; p 349): (n=4) Tres hipoperfusion definida en ambos
lóbulos frontales; en general en todos cierta extension a cortex
temporal y parietal. ?No anormalidad en tálamo, estriado o cerebelo.
El cuarto hipoperfusion leve en zona frontal superior iqda. SPET papel
en dif. de otros síndromes parkinsonianos.
Ver Trabajos PET: hay varios. Dos
subclases.
Arnold-G
SO: J-Neural-Transm-Suppl. 1994;
42: 111-8
AB: In order to in vivo identify
subgroups in eight patients with the clinical diagnosis of progressive
supranuclear palsy (PSP), we have performed 123I-iodobenzamide single photon
emission computed tomography (IBZM-SPECT), a nuclear medicine technique,
to visualize dopamine D2 receptors in vivo, and high resolution (TE/TR
2900/20-90) magnetic resonance imaging (MRI) to evaluate morphological
CNS changes. All patients exhibited similar clinical features including
supranuclear vertical gaze palsy, especially of downward gaze, predominantly
axial rigidity especially in the neck, bradykinesia, instability of balance
with easy falls, and poor response to dopaminergic drugs. Specific striatal
dopamine D2 receptor binding in IBZM-SPECT, as calculated by a basal ganglia
to frontal cortex ratio (BG/FC) was reduced in 5 patients, but normal in
3 patients. In MRI, ?these 3 patients exhibited multiple hyperintense
white matter lesions; 2 of them had no midbrain atrophy. In contrast,
all 5 patients with reduced IBZM binding lacked multiple white matter lesions
in MRI, but 4 of them showed marked midbrain atrophy.This pilot study with
IBZM-SPECT for in vivo imaging of striatal dopamine D2 receptors and T2-weighted
MRI supports published neuropathological findings that clinical signs of
PSP appeared to be due to heterogeneous neuropathology.
AU: Kondo-S; SO: Rinsho-Shinkeigaku.
1994 Jun; 34(6):531-7.
AB: We studied cerebral blood flow
and oxygen metabolism in 6 patients with pure akinesia (PA), 8 patients
with progressive supranuclear palsy (PSP), 16 patients with Parkinson's
disease (PD), and 10 normal control subjects using positron emission tomography
(PET). Regions of interest were studied in the cerebral cortex (the frontal,
temporal, parietal, and occipital lobe), thalamus, cerebellar hemispheres,
cerebellar vermis, and brainstem.
- In patients with PA, regional
cerebral blood flow (CBF) was significantly decreased in the frontal cortex,
thalamus, and brainstem compared with normal control subjects.
- PSP patients showed significantly
decreased CBF in the entire cerebral cortex, thalamus, cerebellar hemispheres,
and brainstem and regional cerebral oxygen metabolic rate (CMRO2) in the
frontal cortex, thalamus, and brainstem, whereas
patients with PD were revealed to be normal in both CBF and CMRO2 values.
In conclusion, a part of patients with PA may be in the early stage of
PSP or may be atypical presentations of PSP whose symptom is only akinesia
judging from the followings.
1) PET findings demonstrated a close similarity between PA and PSP; a pattern
of CBF and CMRO2 decrement especially in the frontal cortex, thalamus and
brainstem. 2) Patients with PA were
shown to have similar pathological changes to PSP in recent studies. 3)
Several authors reported that some PSP cases could show only akinesia initially,
later developing dementia, supranuclear ophthalmoplegia, pseudobulbar palsy,
and nuchal rigidity during their illnesses. |