Journal of Neuro-Ophthalmology, March 1985, Volume 5, Issue 1

Unusual CT findings in progressive supranuclear palsy. Case report.

We report a patient affected by progressive supranuclear palsy who also presented with bilateral internuclear ophthalmoplegia. The computed tomographic (CT) scan showed the previously described radiological picture characteristic of the syndrome and two additional unusual findings. The significance of these findings is discussed and the importance of the CT scan in the diagnosis of progressive supranuclear palsy is emphasized.

f. Clill. NClIrtl-tll'hthalllltll. 5: 59-b I, 19R5. Unusual CT Findings in Progressive Supranuclear Palsy Case Report PAOLO AMBROSETTO, M.D. ANTONELLA BACCI. M.D. Abstract We report a patient affected by progressive supra­nuclear palsy who also presented with bilateral in­ternuclear ophthalmoplegia. The computed tomo­graphic ICT) scan showed the previously described radiological picture characteristic of the syndrome and two additional unusual findings. The signifi­cance of these findings is discussed and the impor­tance of the CT scan in the diagnosis of progressive supranuclear palsy is emphasized. The clinical diagnosis of progressive supranu­clear pals\' mainly relies on the presence of su­pranuclear gaze paralysis. Ocular movement im­pairments other than supranuclear ophthalmo­plegIa are much less frequently present Recently, CT scan has been reported to be of some diag-nustic \'alue in the disease J , Our repurt descnbes a patient affected by PSP who also presented with bilateral internuclear ophthalmoplegIa CT scan showed the previously reported radiological picture of the disease' and two additional unusual findings. Case Report This 7S-Year-old man was seen as an outpatient at the Institute of Neurology of the University of Bologna in October 1982, for a 2-year history of progressive gait disturbance, abrupt falls without loss of consciousness, blurred vision, and fre­quent episodes of forgetfulness and irascibility. At neurologIcal examination, he was fatuous a~d apathetic with masked face and intellectual im­pairment The neck was markedly stiff in exten­sion and there was extrapyramidal hypertonia at the limbs. Ocular examination revealed a severe limitation of upward and downward gaze. In From the Neurological Institute, Univl'rsity of Bologna, School of Medicine, Bologna. Italy March 1985 addition, left and right lateral gaze revealed im­pairment of the adducting eye and dissociated nystagmus of the contralateral abducting eye, suggesting bilateral internuclear ophthalmople­gia. Doll's head maneuver improved the range of vertICal gaze. The tendon reflexes were brisk and the plantar responses were flexor bilaterally. There were no cerebellar signs or sensory loss. EEC, x-ray of the skull, and laboratory data per­tormed elsewhere were reported as normal. The CT scan performed at our Institute 2 months previously showed atrophy of the mesencephalon and quadrigeminal plate, enlargement of the aq­ueduct and third ventricle, and dilatation of the lateral ventricles and cortical sulci (Fig. 1). One week later, the patient developed bronchopneu­monia and died. Pathological specimens were not available. Discussion Our patient showed the clinical features of progressive supranuclear palsy, and the CT find­ings were those described previouslY as charac­teristic of the disease. In addition, he was also affected by internuclear ophthalml)plegia, which is an ocular syndrome characterized by impaired adduction uf une eve and dissuciated nystagmus uf thl' cuntralater,11 abducting eve The syndn)me has a lucalizing diagnustic value and reslI!h from a lesiun uf the nwdi,11 lungitudinal L1sciculus. l1l1 the side uf the impaired ,1dductil)J1. causing inter­ruptiun uf the internuclear mess,1ge cHried by medial lungitudmal L1sciculus bet~\'een the ab­ducens nucll'us ,1nd the nwdi,11 rectus subnucleus. Casl'S uf intl'rnuclear uphthalmuplegia due tl) II1fards: tumurs.'· and dl'n1\'l'linating diseases" " in thl' nwdl,11 lungltudin,11 L1sciculus region have bl'l'n .. ~~:purtl'd prl'viouslY With few excep­tlllns, bil,1kr,1Imternucle,H ophthalmoplegia is rl'ported as virtu,1lly pathognumunic of multiple scll'rosis." Uni- or bil,1kral internuclear ophthal­mopkgla 111 thl' progressive supranuclear palsy has bl'l'n only rarely reported, but is probably marl' frequent than has been hitherto believed. 59 Figure 1. Unenhanced CT scan (magnifICation x2) The mesencephalon IS small and perimesencephaltc CIsterns are prominent. Note the hlpodense bundle in the midbrain (arrowhead) suggesting degeneration In the regIon of the medIal longitudinal fasciculus and the two lucent areas In the regIOn of the hippocampus bilaterally (arrows). In addition to the accepted form of supranuclear gaze paralysis, Mastaglia and Graingerl1 and Perkin et al 12 found this abnormality of ocular movement, respectively in four and in two out of their reported cases of progressive supranuclear palsy. Moreover, at autopsy, Blumenthal and Miller!) found mild loss of myelin in the medial longitudinal fasciculus of their case, even if the patient was not clinically affected by internuclear ophthalmoplegia. Even in the absence of pathological confirma­tion, it is doubtless that our patient was affected by progressive supranuclear palsy and that the medial longitudinal fasciculus as well was in­volved in the pathological process, considering the presence of internuclear ophthalmoplegia. Therefore, it seems reasonable to speculate that the bundle of hypodensity in the mesencephalon could represent the CT expression of the demye­linating process in the medial longitudinal fasci­culus. Degenerative changes in the hippocampal cor­tex, especially in the Sommer's sector are not frequent, but have been reported in the pro­gressive supranuclear palsy. 1 -10 They consist in the presence of Alzheimer's neurofibrillary tan­gles in the cerebral cortex. It is the opinion of Ishino and Otsuki lS that these neurofibrillary tan­gles are a specific manifestation of the progressive 60 supranuclear palsy and are not age-related. In our opinion, the two lucencies located laterally to the mesencephalon could be suggestive of degener­ative changes in the cortical layers of the hippo­campal regions. This CT finding has not been emphasized previously in patients affected by progressive supranuclear palsy; retrospectively, however, it was alsL) present in case 1 of our recent report of patients affected by the Steele­Richardson- Olszewsky syndrome (see Fig. 1 [left] oi rei. 3). In conclusion, we emphasize the use­fulness of the CT scan in studying progressive supranuclear palsy and wish to point the reader's attention to these new radiological findings. References 1. Ambrosettl), P., and Kim, M.: Progressive supra­nuclear palsy. (Letter.) Arch. Nt'l/rol. 38: 672, 1981. ") Haldeman, S, Goldman, JW., Hyde, J., and Pri­bram, H.FW.: Progressive sup;anuclear palsy, Cllmputed tomography, and response to antipar­kinsonian drugs Nt'l/rolllSY 31: 442-445, 1981. 3. Ambrosetto, P., Michelucci, R., Forti, A., and Tas­sinari, c.A.: CT findings in progressive supranu­clear pals\'. f. Comp. Assist. TonlOgr. 8: 406-409, 1984. 4. Gonyea, E.F.: Bilateral internuclear ophthalmople­gIa. Association with occlusive cerebrovascular dis­ease. Arch. Nt'l/ml. 31: 168-173, 1974. Journal of Clinical Neuro-ophthalmology 5. Trl)ost, BT, Martinez, J., Abl'\' A.L., .md lIews, C.R.: Upbeat n~'stagmus.lnd internudl'M uphth'll­mllplegia with brain stl'm ~lil'I1l.I. Ardl. Nt'III'L'I. 37: ol53-ol5b, 14HO o. Baloh, R.W., Yl'l', R.D, .md I !l'llrubi.l. V.: Inll'r­nudear l'phth,llnlllpll'~i.l. I. S.lcc.llil'S .1Ild dissl'­, i.lted nYst.lpnus. :\rdl. Nt'IUtII. 35: olHol-olIN, 147H. 7 B~lll)h, R,\\'., Yel', R.L)" lllh.i I h'nnJbill, V.: Inter­nudeM llphth.llml'pkp,l. II. Pursuit. l'ptll\..inl'tic nYst.lgmus.•md \'l'stibull' 1"IIIM relln ...Irdl. Nt'II­r, I/. 35: ol4ll-ol43. 1478. /\. Smith. !.L...md [I.wid. N.!.: Inll'rtlude.H llphth.ll­ml'plegi. l. T\\'l' 111'\\' clinic.ll si~ns. NCIII'tI/".'\l/ 14: Jll7-3lN. 14 /lol q Schr.wder. I'L .. Cl'l1<'n. I\IN ...md Cl,lm.m. W.. B,I.Ill'r,11 inll'rtluck.H l'phth.llnlllpll'gia .lSSIll'iall'd \\'Ith il'Urth Yentricular epidt'rml"d tunwL f. Nt'I/­f, I,'IrI: 54: ol1l3-ol115. 14/\ I Ill. Fllrd~ CS. Cruz. I. BilkLI., Laster. W., and \\'hith,'. DR.: Bi1.ltt'ral internudeM l'phthalmuple­~,. l In C,lfCillllm.ltl'uS meningitis. f. Clill. NCI/Ttl­' 11'llth'IIIII"/. 3: 127-130. 1483. II. \Iastagli.l. F.L.. .md Grainger. M.R.: Internudear ,'phthalnlllplepa in pfll~ressiYe supranudear p.llsY I .\·,'IIr,'/. St'!. 25: 303-308. 1975. March 1985 Ambrosetto, Bacci 12. 1\·r\..lll, GD., Lees, A.J., Stern, G.M., and Kocen, RS.: l'rubkms in the diagnosis of progressive su­pr. lnudear palsy (Slede-Richardson-Olszewski .syndrume) CIIII f. NCllrol. Sci. 5: 167-173. 1978 13. Illunwnthal, If. and Miller, c.: Motor nuclear in­\'" Ivenll'nt in progressive supranuclear palsy. Arch. N"llrol. 20: 302 307. IlJ69. j4 Ikhrm.m, S, Clrwl, J.D., Janota, I., and Matthews, w,s: l'wgn'ssive supranuclear palsy Clinico­p. lthological study of fuur cases. Brllill 92: 663- h711. 1404 . 15. !shilll', II, '.ll1d Otsuki, S.: Frequency of Alz­heimer's neurofibrillary tangks in the cerebral cor­ll'x in pwgressive supranuclear palsy (subcortical .H~yrophili('dystrophy). f. NCI/rol. Sci. 28: 309-316. 1970. Ih. Mannen, T.. Toyokura, Y., Miyatake, T, Mohn, M" and Yokoyama, T: Progressive supranuclear palsy. Report of an autopsied case. Ad". NCI/r(l/ 16: 497-503,1972. Writc {M rcprillf~ to: DL Paolo Ambrosetto, Clinica Neurologica, Via Ugo Foscolo 7, 40123 Bologna, Italy. 61