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Natural History, MRI Morphology, and Dopamine Receptor Imaging with 123IBZM-SPECT

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Natural History, MRI Morphology, and Dopamine Receptor Imaging with 123IBZM-SPECT J7ournal ofNeurology, Neurosurgery, and Psychiatry 1994;57:1047-1056 1047 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.9.1047 on 1 September 1994. Downloaded from Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT J B Schulz, T Klockgether, D Petersen, M Jauch, W Muiller-Schauenburg, S Spieker, K Voigt, J Dichgans Abstract of the intermediolateral cell columns of the Sixteen patients with a clinical diagnosis spinal cord. All of these changes are not neces- of probable multiple system atrophy sarily found in each patient with MSA. In (MSA) were examined clinically by MRI some patients, degeneration affects mainly the and by 523I-iodobenzamide single photon basal ganglia, whereas in others the ponto- emission computed tomography (IBZM- cerebellar system or the spinal cord are more SPECT). The clinical records of another affected. Therefore, evidence of cell degenera- 16 patients were also analysed retrospec- tion and gliosis in at least three of the following tively. On the basis of their clinical pre- anatomical structures is thought to be suffi- sentation, patients were subdivided into cient for the neuropathological diagnosis of those with prominent parkinsonism MSA: striatum, substantia nigra, Purkinje (MSA-P, n = 11) and those with promi- cell layer of the cerebellar cortex, pontine nent cerebellar ataxia (MSA-C, n = 21). nuclei, inferior olives, and intermediolateral Autonomic symptoms were present in all cell columns of the spinal cord.'4 Striatum or patients and preceded the onset of motor substantia nigra must be one of these areas.5 symptoms in 63% of patients. Calculated By contrast with idiopathic Parkinson's syn- median lifetime and the median time to drome, there is no Lewy body pathology in become wheelchair bound after onset of MSA. Rather, a recent study had reported the disease were significantly shorter for presence ofglial cytoplasmatic inclusion bodies MSA-P than for MSA-C (lifetime: 4 0 v in MSA.6 The specificity of this finding 9-1 years; wheelchair: 3-1 v 5'0 years) remains to be proved in further studies. suggesting a better prognosis for cerebel- The clinical presentation of patients with lar patients. A significant loss of striatal MSA is highly variable, at least at the onset of dopamine receptors (below 2 SD thresh- the disorder: patients may present with old) was detected by IBZM-SPECT in parkinsonism, with a cerebellar syndrome, 63% of the patients (56% below 2 5 SD with progressive autonomic failure, or with threshold). There was no difference variable combinations of these syndromes. between patients with MSA-C and those Usually parkinsonian symptoms respond only with MSA-P in the proportion with sig- poorly to levodopa. Only a minority of nificant receptor loss and the extent of patients with MSA are correctly diagnosed dopamine receptor loss. Planimetric during life due to equivocal clinical sympto- MRI evaluation showed cerebellar and matology and the lack of definite diagnostic http://jnnp.bmj.com/ brainstem atrophy in both groups. tests. Accordingly, some patients with MSA Atrophy was more pronounced in are simply diagnosed as having Parkinson's Department of patients with MSA-C than in those with disease. Other patients are categorised under Neurology MSA-P. Pontocerebeliar hyperintensities the umbrella terms Parkinson-plus syndrome J B Schulz and putaminal hypointensities on T2 or atypical parkinsonism. Patients with a com- T Klockgether S Spieker weighted MRI were found in both groups. bination of parkinsonism and autonomic fail- J Dichgans Pontocerebellar signal abnormalities ure are often referred to as having Shy-Drager on October 1, 2021 by guest. Protected copyright. Department of were more pronounced in MSA-C than in syndrome. On the other hand, MSA may be Neuroradiology MSA-P, whereas the rating scores for the underlying pathology in patients with D Petersen area but not for intensity of putaminal prominent cerebellar ataxia who received the K Voigt abnormalities were higher in MSA-P. diagnosis of sporadic olivopontocerebellar Division ofNuclear Medicine, MRI and IBZM-SPECT provide in vivo atrophy or idiopathic cerebellar ataxia during Department of evidence for combined basal ganglia and life. In an attempt to form a rational basis for Radiology, University pontocerebellar involvement in almost all the intra vitam diagnosis of MSA, Quinn7 pro- ofTubingen, Hoppe- patients in this series. Seyler-Strasse 3, posed clinical criteria for the diagnosis of D-72076 Tubingen, probable MSA; these include the presence of Federal Republic of (T Neurol Neurosurg Psychiatry 1994;57: 1047-1056) parkinsonism or cerebellar ataxia and severe Germany M Jauch symptomatic autonomic failure without W Muller-Schauenburg dementia and downgaze palsy. Dependent on Correspondence to: The term multiple system atrophy (MSA), whether parkinsonian or cerebellar symptoms Dr T Klockgether, first used by Graham and Oppenheimer in predominate the diagnosis is parkinsonian Department of Neurology, University of Tubingen, 1969,1 refers to a sporadically occurring adult (MSA-P) or cerebellar (MSA-C) (probable) 72076 Tubingen, Germany. onset neurodegenerative disease. Neuro- MSA. Received 29 March 1993 pathologically, MSA is characterised by the In recent years, modem imaging methods and in final revised form 15 November 1993. presence of olivopontocerebellar atrophy, such as MRI,'" SPECT," '3 and PET'4 15 Accepted 26 November 1993 striatonigral degeneration, and degeneration have increasingly been used in neurodegenera- 1048 Schulz, Klockgether, Petersen, Jauch, Midler-Schauenburg, Spieker, Voigt, Dichgans J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.9.1047 on 1 September 1994. Downloaded from tive diseases. Although these methods have standing. Orthostatic reduction in systolic also been applied to patients with MSA, their blood pressure of 30 mm Hg or more without diagnostic value in MSA remains uncertain. a sufficient increase of heart rate was consid- The reported abnormalities, such as putami- ered orthostatic hypotension. On the day of nal iron accumulation or loss of dopamine the clinical investigation MRI and IBZM- receptors, are found in other parkinsonian SPECT were performed. Two patients in syndromes. Also, most studies are restricted whom MRI could not be performed due to to patients with MSA who have parkinsonian claustrophobia or a metallic implant received symptomatology, whereas patients with cere- a high resolution cranial CT examination. bellar symptoms have attracted much less Those patients with probable MSA who attention. were not seen personally (n = 16) were In this study, we report clinical, MRI, and included only in the outcome evaluation. For "23I-iodobenzamide (IBZM) single photon these patients, age at onset of first notable SPECT data from a series of 16 patients with motor symptom, clinical type (parkinsonian v probable MSA of either parkinsonian (MSA- cerebellar), duration after onset of motor P) or cerebellar (MSA-C) type. The aim of symptoms until they became wheelchair the present study was twofold. Firstly, we bound, and age at death were determined wished to study the natural history and prog- from the clinical records. In many patients nosis of MSA and to compare MSA-C and autonomic symptoms antedated motor symp- MSA-P. Secondly, we wished to study the toms by several years. Autonomic symptoms abnormalities detected by MRI and IBZM- were often vague and of undetermined onset, SPECT in patients with MSA. In particular, however, so that it turned out to be impossi- we were interested in whether these imaging ble to use them for exact temporal definition procedures can identify abnormalities that are of onset of disease in the 16 patients studied common to all patients with MSA or whether retrospectively. they yield different results in cerebellar and parkinsonian patients. MRI MRI was performed with a 1'5 T supercon- ducting system (Magnetom, Siemens AG, Patients and methods Erlangen, Germany) with a circularly PATTENTS polarised head coil. Data were displayed on a The clinical records of all patients with 256 x 256 matrix. All patients were investi- parkinsonian symptoms (n = 850) and all gated by a standard examination programme patients with cerebellar degenerative disease that included sagittal and axial Ti weighted (n = 217) referred to our department between images (spin echo, TR = 600 ms, TE = 15 1983 and 1991 were reviewed. Of these, 32 ms, slice thickness = 4 mm), and axial pro- fulfilled all of the following four diagnostic cri- ton-density and T2 weighted images (dual teria for probable MSA: (a) progressive cere- spin echo, TR= 2100 ms, TE = 45 + 90 bellar ataxia and/or non-responsive or poorly ms). To improve slice profiles two subsequent responsive levodopa parkinsonism without sets of measurements of each sequence were evidence for a focal or non-focal symptomatic made, each with an interslice gap of 4 mm. origin of the disorder; (b) severe symptomatic Both sets of measurements together gave a autonomic failure with at least postural syn- complete series of images without a gap. cope or presyncope and/or pronounced uri- Special care was taken to position the head http://jnnp.bmj.com/ nary incontinence or retention not due to and neck symmetrically in the head coil. other causes; (c) absence of any neurodegen- Twenty-four age and sex matched healthy erative disorders in relatives and no evidence persons served as controls (mean age 58-0 of consanguinity of parents; (d) absence of (15-6) years). Neurological examination of dementia according to DSM-III R criteria, these persons was normal and routine evalua- generalised tendon areflexia, or predominant tion of their MRIs gave no pathological find- downgaze supranuclear palsy. Based on their ings. clinical presentation, patients were subdivided Quantitative MRI evaluation of cerebel- on October 1, 2021 by guest. Protected copyright. into two groups, one with predominant cere- lum, pons, brainstem, and spinal cord was bellar symptoms (MSA-C, n = 21) and performed under blinded conditions. Regions another with predominant parkinsonian pre- of interest of any form, location, and size sentation (MSA-P, n = 1 1).
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