Table 3. Parkinson’s Disease Oxidative Phosphorylation Enzymology ~~
~~
Controls
Parkinson Disease Patients
Complex
Assay
N
XkSD
5%
I I
NADH-DB NADH-CYTC DBH,-CYT C succ-CYTC CYT C OXIDASE
11 11 11 11
194 2 60 229 2 149 1 9 5 2 r 700 605 t 164
54 0 323 223
9 11
1426 i- 433 1615 k 309
373 896
+ 111
111 I1 IV
+ 111
Fr SONICATED
2
3
4
5
6
16 8 633 188
20 13 895 259
27 16 1206 511
74 80 2110 462
92 75 1164 284
174 126 2009 490
1482 63
758 648
1772 843
1323 941
326 677
1026 1054
1
NADH-DB = NADH-n-decyl coenzyme Q oxidoreductase; NADH-CYT C = NADH-cytochrome c oxidoreductase (rotenone sensitive fraction); DBH2-CYT C = reduced n-decyl coenzyme Q-cytochrome c oxidoreductase; CYT C OXIDASE = cytochrome c oxidase; ET = freeze-thaw.
N, mean 2 SD, 5% confidence level for older conrrols (age: 45-69 yr): complex I + 111 = 6, 135 Tt 66, 0; and complex I1 606 +- 165, 148. Enzyme activity is reported in nanomoles of substrate/minute/rng mitochondrial protein.
We reasoned that, if such patients could be found among our referrals for OXPHOS determination, then these factors alone probably do not account for the OXPHOS defects in Parkinson’s patients. The inadvertent addition of the patient data to the control group had a negligible effect on the reported mean and standard deviation. Hence, our conclusion that defects of OXPHOS are found in skeletal muscle from some patients with PD is unchanged. However, additional studies are required if we are to understand fully the significance of the observed association between OXPHOS deficiency and PD. ‘Department of Genetics and Molecular Medicine ?Department of Neurology Emory University School of Medicine Atkanta, GA
Increased Signal in Basal Gangha and-White Matter on Magnetic Resonance Imaging in Creutzfeldt-Jakob Disease Kiyoshi Yamamoto, MD, and Mitsunori Morimatsu, M D Milton and colleagues El) reported a biopsy-diagnosed patient with Creutzfeldt-Jakob disease (CJD) showing increased
114 Annals of Neurology Vol 32 No 1 July 1992
+ 111
=
6,
signal intensity in the basal ganglia on magnetic resonance imaging (MRI). Recently we reported serial changes on cerebral MRI in a patient with CJD E2). In the early stages, MRI showed several high-intensity lesions in the putamen and caudate bilaterally and mild periventricular high intensity (PVH) on T2-weighted images. After three months, MRI revealed progression of cerebral atrophy, ventricular dilatation, moderate PVH, and increased signal intensity in putamen and caudate bilaterally. In the terminal stage, MRI showed diffuse marked hyperintensity in the white matter and basal ganglia. We agree with Milton and colleagues that in a patient with a rapidly progressive dementia, the MRI demonsrration of bilateral increased signal intensity on long TR images in deep gray structures should suggest the diagnosis of CJD. In addition, we believe that the white matter involvement of CJD may develop late following degeneration and atrophy of gray matter. Department of Neurology Yamaguchi University School of Medicine Ube,Japan References 1. Milton WJ, Atlas SW, Lavi E, et al. Magnetic resonance imaging of Creutzfeldt-Jakob disease. Ann Neurol 1991;29:438-440 2. Yamamoto K, Fukusako T, Nogaki H , et al. A case of Creutzfeldt-Jakob disease. Serial changes of cerebral magnetic resonance imaging. JJMR 1991;11:83-87