I SOTOPIC cisternography obtained

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1 At(;USr, 1972 CEREBELLAR ATROPHY: A CISTERNOGRAPHIC AND PNEUMOENCEPHALOGRAPHIC ANALYSIS By ERNST-PETER STRECKER, M.D.,* FREDJ. HODGES, III, M.D.,f and A. EVERETTE JAMES, JR., SC.M., M.D4 I5ALTIMORE, I SOTOPIC cisternography obtained injection of a radiopharmaceutical into the subarachnoid space gives valuable information regarding anatomical and functional abnormalities of cerebrospinal fluid (CSF) space and dynamics. CSF imaging has proved useful in detecting various intra-and extracranial congenital and acquired disorders associated with localized CSF compartment enlargement and in differentiating the various forms of hydro- 4,5 Also, cisternographv max be of predictive value in determining which patients may benefit from a CSF diversionarv shunt.1#{176} This report describes 6 patients with cerebellar atrophy who underwent cisternography, because of the clinical suspicion of normal pressure hydrocephalus (NPH). The prognosis in patients with cerebellar degeneration is, in general, poor. However, in normal pressure hvdrocephalus, the prognosis is often good after CSF diversionary Therefore, it is important to separate these 2 entities. The cisternographic pattern in cerebellar degeneration is correlated with pneumoencephalographic findings and the value of CSF imaging as a screening procedure is discussed. MATERIAL AND METHOD The 6 patients were in a series of more than 400 cisternographies performed at the Johns Hopkins Hospital. All 6 patients were in the age range from 50 to 70 years. They manifested ataxia, gait problems, and dementia. Because of these MARYLAND symptoms and neurologic findings, the clinical diagnosis of normal pressure hydrocephalus was considered and a cisternogram obtained as part of the evaluation. However, in all of these patients, cerebellar degeneration with various dinical etiologies was documented (Table i). The routine method for cisternography for evaluation of suspected hydrocephalus in our laboratory consists of a lumbar subarachnoid injection of an appropriate radiopharm aceu ti cal 1 hu m an serum albumin (HSA), Tc99 HSA, Yb 69 diethylenetriamine pentaacetic acid (DTPA), or In DTPA). Images are obtained in the anterior, posterior and lateral positions at 2, 6, 24 and 48 hours. Occasionally 72 hour images are made. Either a rectilinear scanner or a scintillation camera is employed. The normal progression of the radiopharmaceutical is from the lumbar area to the basal cisterns and then through central and peripheral pathways to the parasagittal region (area of the arachnoid granulations). TABLE CLINICAL ETIOLOGY FOR CEREBELLAR ATROPHY Alcoholism 2 Tabes dorsalis I Olivopontocerebellar degeneration I Cerebellar infarction associated with cerebral arteriosclerotic disease I Unknown Associated with Paget s disease Acoustic neuroma Normal Pressure hydrocephalus * Fellow, Radiological Research, Department of Radiology, Johns Hopkins Medical Institutions, Baltimore, Maryland. t Associate Professor in charge of Neuroradiology, Department of Radiology, Johns Hopkins NIedical Institutions. Director, Laboratory for Radiological Research, Department of Radiology and Radiological Sciences, Johns Hopkins Medical Institutions, Baltimore, 1\ilaryland. Supported by grant M James A. Picker Foundation (NAS National Academy of Science. NIH-Training grant Tos GM0I328-o7. Diagnostic Radiology Training Grant. I 760

2 \o. 115, No. Cerebeliar Atropily No ventricular entry of radiopilarmaceutical should be preseilt. File parasagittal radiopil arm aceu tical concen tra tion w m11 normally occur by 24-4 ilours. Abnormal flow patterns consist of: ( i) cessation of radioph arm aceu tical movement (momentary or for mans- hours) at a point of CSF space obliteration; (2) entry of the radiopharmaceutical illto tile lateral ventricles witil clearing by 24-4 ilolirs ( com m ii nicati ng h vdroceph alus wi tilotl t stasis ) ; (3) ventricular entry of the radiopharmaceutical without clearing ( normal pressure il\-drocephalus) ; () localized collection of radiopilarmaceuticai outside the ventricular system; and (5) generali zed dela. mn rad ioph arm aceu tical flow. Fi ye )atieflt5 u Ilderwen t pne1ime11- cephalogra)h\ ( PEG) which i ncluded au totomograms of tile posterior fossa. In I patient pneumoencephalograpil\ was not performed because tile abnormalities present on the cisternographic study, the associated l)hvsical findings, and clinical history were adequate to establisil the diagnosis ofcerebeliar degeneration. RESULIS All of the patients under examination showed abnormal cisternograpilic patterns. Tilere was an increased amount of radiopharmaceutical in tile posterior fossa on the 2 and 6 hour images; especialls in the area of tile cisterna magia and supracerebellar cisterns (Fig. i, 1 and B). In 2 patients increased concentration of the radiopharmaceutical in the posterior (OSSa persisted on the 24 hour images (Fig. 2, 1 and B). In the other I)atients the radioactivity appeared uniforml- distribu ted throughout the subarachnoid CSF space on A -, -) --- _ crease otthe -rat I IG I (A) \orm il c tse Right I ster ii 6 hour cis foss it is jresen ternographic view after lumbar subarachnoid in- certbellum anl iii-,i jection of i mc Yb 9 1)TPA. Radiopharmaceutical the surroundini- - is present in the basal cisterns, but is not seen in isterns iri=t1ii the lateral ventricles (activity over quadri- pnt, ) II I,, I 0I I = 44 - #{149} I #{149}, = I. - TIIII II 4I iti

3 762 E.-P. Strecker, F. j. Hodges, III, and A. E. James, Jr. AU;tsr, 1972 I IG. 2. (A) Right lateral and (B) posterior 24 hour views in a chronic alcoholic show increased accumulation of the radiopharmaceutical in the posterior fossa. Note the amount of radiopharmaceutical over the parasagittal area at 24 hours-a normal feature. the delayed images but was accumulated in the posterior fossa on the initial images. In patients entry of the radiopharmaceutical into the lateral ventricles was observed. There was stasis of the radiopharmaceutical within the lateral ventricles during the hour period in 2 patients. They had obvious pneumoencephalographic and cisternographic signs of normal pressure hvdrocephalus. The first had tabes dorsalis, the second Paget s disease and a right acoustic neuroma. In the remaining 2 patients, ventricular entry with clearing was observed. In the 5 patients in whom pneumoencephalograms were obtained, signs consistent with decreased cerebellar volume were present. Several radiographic signs in combination must be present to establish tile diagnosis of cerebellar atrophy.28 12,13 DISCUSSION The hereditary types of cerebellar atrophy manifest usually in earls adult life. They will not be discussed in this communication because all patients in this series had sporadic or aquired lesions of the cerebellum. Olivopontocerebellar atrophy occurs in later life and is sporadic or familial. Parenchvmatous cerebellar atroph is characterized by loss of the cerebellar cortex, especially degeneration of the Purkinje cells. It is caused by antecedent illness, such as carcinoma elsewhere (usuallv lung), tabes dorsalis and chronic alcoholism.3 #{176} Focal decrease in the volume of the cerebellum is seen with vascular disorders, following posterior fossa stirger, or due to osseous developmental anom 8 It is difficult to distinguish these disorders clinically. All are characterized by s mptoms of cerebellar deficiency, mainly dysarthria, ataxia with tremor of the limbs, and ataxic gait. Mental deterioration may occur in the later stages.3 Normal pressure hydrocephalus will manifest these same symptoms and lead to clinical confusion; especially if associated with only minimal dementia, long tract and cerebellar signs as described by Heinz et al.4 The clinical recognition of degeneration of the cerebellum is rarely certain, unless the clinical course is a protracted one.3 It is usually possible to detect cerebellar atrophy with certainty from the pneumo_ encephalographic appearance.8 3 The diagnosis cannot be based upon the size of the cisterna magna alone, for great variation in the size of this space has been en-

4 Voi. 115, No. Cerebellar Atrophy 763 countered in normals and patients free of cerebellar disease912 3 (Fig. 3/1). The range of normal variation of other CSF con taming spaces, except for the cerebellar sulci, may also be great.9 Therefore, several pneumoencephalographic signs in association are necessary to establish this diagnosis. Enlargement of the foramen of Magendie and the vallecula is much more commonly encountered in cerebellar degeneration than as a normal anatomic variant. The depth of the superior cerebellar cisterns varies greatly, but an extensive collection of air at pneumoencephalography over the upper part of the cerebellum and wide sulci signify atrophy. Fourth ventricular size as measured on pneumoencephalograms varies greatly in the normal with a range of cm. transversely and cm. in its sagittal diameter) Fourth ventricular enlargement is significant (Fig., /1 and B), especially in combination with other radiographic signs of cerebellar atrophy. 8 3 Since a radiopharmaceutical imaging study does not have the anatomic resolution of the PEG and, therefore, does not optimally delineate small anatomic variations, the results of this examination cannot be as specific. One is able, however, to demonstrate enlarged cisterns and to anatomically identify them.7 As previously noted an enlarged cisterna magna alone (Fig. 3B) is not specific for the diagnosis of cerebellar atrophy. Several signs must be present to increase the probability of this diagnosis. In these patients with cerebellar atrophy, there was increase of radioactivity \ - t lateral cisternogram at 6 hours (lumbar injection of mc Yb 1)TPA). Ventricular entry of the radiopharmaceutical consistent Wi th communicati ng h ydrocephalus is present. The increased amount of radiopharmaceutical in the posterior compartment of the posterior fossa is due to a large cisterna magna, as demonstrated by pneumoencephalography. (B) Pneumoencephalogram (erect autotomogram). in the supracerebellar CSF space, in the lateral recesses of the pontine cisterns, and in the cisterna magna in combination. Thus, the CSF imaging study can be used only as a diagnostic screening test; for a more definitive diagnosis a PEG must be performed. It is unlikely that cerebellar degeneration occurs secondary to CSF pathway obstruction over the cerebral hemispheres. Two patients in whom both cerebellar atrophy and ventricular entry of the radiopharmaceutical with stasis were documented had other associated disorders. These other abnormalities were felt to be responsible for the changes in the posterior fossa. The first

5 764 E.-P. Strecker, F. J. Hodges, III, and A. E. James, Jr. Atu SF, G.. (A) This patient had arteriosclerosis and clinicall sufkred a cerebellar infarction. The pneumoencephalogram demonstrates an enlarged fourth ventricle and focal atrophy of the midportion of the cerebellar vermis shown by the enlarged folia. (B) On the ( hour lateral view of the cisternogram an abnormally large amount of radiopharmaceutical is seen in the posterior fossa. Ventricular entry with clearing of the radiopharmaceutical b 24 hours consistent with cerebral atrophy was seen on serial images. had tabes dorsalis, tile second had both Paget s disease with osseous deformit of tile base of the skull and a tumor of the rigilt eigiltil cranial nerve. B cisternograpil\- we can detect cerebellar atrophy, even wilen present witil normal pressure h drocephalus or generalized cerebral atrophy. In patients with normal pressure hdrocephaltis the cisternogram demonstrates radiopharm aceutical entry illto enlarged lateral ventricles witil stasis (ventricular retention). In patients witil generalized cerebral atrophy, ventricular entry can be observed. There is 110 stasis and concentration of tile radio- I)harmaceutic1l in the parasagittal area occurs by ilour5. In patients witil neurologic signs and clinical suggestions of either cerebellar atrophy, normal pressure hvdrocepilalus or a combination of tile 2, it is possible to recognize each entity by cisternographv. Other diseases can mimic cerebellar degeneration by an increased amount of radioactivity in the posterior fossa. Suboccipital injection of the radiopharmaceutical into the cisterna magna and subdural injection are tecilnical causes. Ihere is also all increased amoullt ot radioactivity on cisternograpilv in the posterior fossa of patients witil Paget s disease, achondroplasia, communicating types of Dandy- \Valker cysts, or extraventricular posterior fossa cysts, cerebellar porencephalic c sts, and meningoceles of the posterior fossa. A pneumoencephalogram is necessar to identif mans of these causes of abnormal p05- tenor fossa CSF collections. The amount of radiopharmaceutical that abnormally concentrated in the posterior fossa in these patients witil cerebellar atropilv correlated well with tile findings demonstrated by pneumoencephalographv. None of the patients witil cisternographic findings or cerebellar atrophy had a normal pneumoencephalogram. Cisternographv is abnormal in patients with cerebellar atrophy and is useful as a screening procedure. As in the diagnosis of normal pressure hvdrocephalus and localized enlargement of the CSF space, it is valuable not only as a screening procedure,

6 VOL. 115, No. Cerebellar Atrophy 765 but as a complementary diagnostic stud\ to pneumoencephalography which should be performed for confirmation and anatomic detail. SUMMARY The cisternographic appearance of 6 patients with cerebellar atrophy is reviewed. These findings are correlated with those found at pneumoencephalography in 5. The analysis suggests that one cannot, at present, specifically diagnose cerebellar atrophy by CSF imaging, but that it offers excellent promise as a screening procedure..a. Everette James, Jr., M.D. Department of Radiology The Johns Hopkins Hospital Baltimore, Maryland REFERENCES I. BENSON, D. 1., LEMAY, M., and P#{149}ATTEN, D. H. Diagnosis of normal pressure hydrocephalus. New England 7. Med., 970, 283, BETZ, H. S. Korrelation der klinischen Symptomatik mit dem radiologischen Befund beim Pneumotomogramm der hi nteren Sch#{228}delgrube. Radiologe, 1969, 9, BRAIN, L., and WALTON, J. N. Progressive cerebellar degeneration. In: Brain s l)iseases of the Nervous System. Oxford University Press, London, 1969, HEINZ, E. K., DAvIS, D. 0., and KARL, H. E. Abnormal isotope cisternography in symptomatic occult hydrocephalus: correlative isotopic neuroradiological study of 130 subjects. Radiology, 1970, 95, JAMES, A. E., I)ELAND, F. H., HODGES, I. J., III, and WAGNER, H. N., JR. Cisternography: its role in evaluation of normal pressure h>drocephalus. 7.A.M.A., 1970, 213, JAMES, A. E., HARBERT, J. C., I)ELAND, 1. H., MCCULLOUGH, I). C., HODGES, I. J., and WAGNER, H. N. Localized enlargement of cerebrospinal fluid space demonstrated by cisternograph y. Neuroradiol., 7 I, 2, 184- I JAMES,.A. E., JR., l)eland, l. H., HODGES, I. J., 111, and WAGNER, H. N., JR. with assistance of W. A. North. Cerebrospinal fluid (CSF) scanni ng: cisternography. AM. J. ROENTGENOL., RAD. THERAI Y & NUCLEAR MED., 970, 110, LEMA\-, M., and AIIRAMOWICZ, A. Pneumoencephalographic findings in various forms of cerebellar degeneration. Radiology, I 965, 85, LILIEQUIST, B. SuI,arachnoid cisterns. Ada radiol., 1959, SU))l. i MCCULLOUGH, D. C., HARBER-I, J. C., l)1chir0, G., and OMMAYA, A. E. Prognostic criteria for CSI shunting from cisternography in communicating hydrocephalus. Neurology, i 970, 20, II. MERRITT, H. H. Cerebellum: degenerative and heredodegenerative diseases. In: A Textbook of Neurology. Lea & 1 ebiger, Philadelphia, 1967, pp I. 12. ROBER-ISON, E. G. Diagnosis ofcerebellar atrophy. In : Pneumoencephalography. Charles C Thomas, Publisher, Springfield, Ill., 1967, pp TAVERAS, J. M., and WOOD, E. H. Cerebellar hypoplasia. In: 1)iagnostic Neuroradiology. Williams & Wilkins Company, Baltimore, 1964, PP

7 This article has been cited by: 1. F. Bianco, L. Bozzao, C. Colonnese, L. Fantozzi The value of computerized tomography in the diagnosis of cerebellar atrophy. The Italian Journal of Neurological Sciences 4:1, [CrossRef] 2. P. Kennedy, M. Swash, I. G. Wylie The clinical significance of pneumographic cerebellar atrophy. The British Journal of Radiology 49:587, [CrossRef]