Postgraduate Medicine ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20 Temporal arteritis presenting as ataxia and dementia Keith N. Shenberger, John G. Meharg Jr & C. Darrell Lane To cite this article: Keith N. Shenberger, John G. Meharg Jr & C. Darrell Lane (1981) Temporal arteritis presenting as ataxia and dementia, Postgraduate Medicine, 69:2, 246-249, DOI: 10.1080/00325481.1981.11715694 To link to this article: http://dx.doi.org/10.1080/00325481.1981.11715694 Published online: 07 Jul 2016. Submit your article to this journal View related articles Citing articles: 8 View citing articles Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [Australian Catholic University] Date: 10 August 2017, At: 16:33 Temporal arteritis presenting as ataxia and dementia Keith N. Shenberger, MD, John G. Mehug, Jr, MD, C. Darrell Lane, MD Downloaded by [Australian Catholic University] at 16:34 10 August 2017 POSTGJL\DUATE MEDICINE invites submission of brief case reports, especially those related to ambulatocy medical care. mustrations and references should be included only when essential. CASE REPORT 246 A 66-year-old woman had had chest pain and shortness of breath for one week. Her symptoms were associated with atrial fibrillation and were unresponsive to attempts at diuresis. The fibrillation and congestive heart failure had been successfully treated with digoxin and diuretics in the past, but this time the dyspnea failed to respond to diuretics ~md the patient was admitted to Reading Hospital and Medical Center, West Reading, Pennsylvania The patient denied having cough, hemoptysis, leg pain or swelling, fever, chills, or diaphoresis. She was in moderate respiratory distress but without cyanosis. Mild jugular venous distention was present. The chest was clear. Cardiac examination showed an irregular rhythm with an 5:J gallop. Trace ankle edema was present. Her temperature was 36.4 C (97.5 F); radial pulse rate 103/min and irregula.rly irregular; blood pressure 1B4/98 mm Hg; and respirations 24/min and unlabored. The patient was oriented, and there were no focal neurologie findings. Laboratory values included: hemoglobin 14.5 gm/dl, hemato- crit 42%, WBCs 12,200/cu mm with normal differentiai, platelets 306,000/cu mm, and ESR (Westergren) 85 mm/hr. Results of coagulation studies were normal, as were arterial blood gas values with the patient breathing room air. Values on multichannel biochemical analysis (SMA-12) were within normallimits, except for urie acid (14.9 mg/dl) and glucose (210 mg/dl). The BUN level was 14 mg/dl and creatinine 1.1 mg/ dl An ECG revealed atrial fibrillation with rapid ventricular response and diffuse, nonspecific ST-and T-wave changes. A chest x-ray film was interpreted as normal. but a lung scan indicated a high probability of pulmonary emboli. Anticoagulation was achieved with heparin; dyspnea and chest pains slowly resolved. Multiple other problems ensued, including transient azotemia, urinary tract infection, and nausea and vomiting. These conditions slowly resolved after administration of fluids and antibiotics and withdrawal of other medication. On the tenth hospital day. the patient was noted to have normochromic normocytic anemia, with a hemoglobin level of 10.9 gm/dl and a hematocrit value of TEMPORAL ARTERITIS•VOL 69/NO 2/FEBRUARY 1981/POSTGRADUATE MEDICINE Downloaded by [Australian Catholic University] at 16:34 10 August 2017 Lumbar puncture revealed clear, colorless fluid with pressure of 150 mm H2 0. CSFstudies showed a cell count of 10/cu mm, aU lymphocytes; protein level of75 mg/dl; and glucose level of74 mg/dl. (Simultaneous blood glucose level was 151 mg/dl.) Microbiologie studies gave negative results. The patient remained somnolent disoriented, ataxie, and confusedoverthenextweekM~s Figure 1. Langhans' multinucleated giant cell in association with fragmented internai elastic lamina. (Verhoeff's stain x100) 31.5%. Studies for hemolysis and bleeding were negative, and the patient was felt to have the anemia of chronic disease. She continued to improve and discharge was contemplated. However, with increasing ambulation the patient was noted to be vezy unsteady on her feet a condition which had not occurred with any of her prior hospitalizations. At the same time, she rapidly became progressively lethargie and disoriented, to the point of being so confused at night that restraints were required. Examination by a neurologie consultant revealed only hypoactive deep tendon reflexes, truncal ataxia, and a left carotid bruit. The patient specifically denied having visual problems, headaches, vertigo, weakness, or sensozy loss. Findings on skull films and computed tomographie and radionuclide brain scans were within normallimits. An EEG showed a mild, diffuse symmetric increase in slow-wave activity. time, renal liver, and thyroid function; electrolyte values; and arterial blood gas levels were checked and found to be normal; ESR was 105 mm/hr. Tests for antinuclear antibodies, rheumatoid factor, lupus etythematosus cells, and czyoglobulins were negative. Findings on serum protein electrophoresis were normal. Temporal arteritis as a possible explanation for her symptoms was suggested, even though severa! examinations of bath temporal arteries were completely unrevealing-normal pulsations and no redness, tendemess, or nodules. Bilateral temporal artezy biopsy was performed. with a specimen being taken just anteri or to the ears on the sides of the face for convenience and cosmetic reasons, since no specifie abnormality had been palpated. Hematoxylin and eosin continued VOL 69/NO 2/FEBRUARY 1981/POSTGRADUATE MEDICINE•TEMPORAL ARTERITIS 247 Downloaded by [Australian Catholic University] at 16:34 10 August 2017 CASE REPORT CONTINUED stain revealed subintimal proliferation, with foci of round œils and giant œils in the media and adventitia Fragmentation of the internai elastic lamina was seen on special stains (figure 1). These findings were consistent with a diagnosis of typical giant œil arteritis involving the temporal arteries. Administration of prednisone, 60 mg/ day. was begun. The next moming. the patient was remarkably better, alert. and completely oriented. Over the next two days. the ataxia cleared and she began to walk and think normally. On repeated questioning, she denied having headaches. visual disturbanœ, jaw claudication, or symptoms of polymyalgia rheumatica The temporal arteries were nontender with normal pulsations. The patient was never febrile. On the second day of prednisone therapy. ESR was 72 mm/hr and hemoglobin level was 12.2 mg/ dl. The patient was discharged on the 36th hospital day feeling weil. with foilow-up care to be given by her intemist and ophthalmologist Discussion Temporal arteritis, or giant œil arteritis of the aged as it has been called, is a disease of protean manifestations, usually occurring after age 50, and more 248 commonly in women. Weilrecognized signs and symptoms include fever. headache, weight loss, anorexia malaise, and nodular temporal arteries that are painful. tender. and swoilen. 13 Multiple other disorders may be seen initially or later during the course of the illness, including polyarthralgia and polymyalgia4 ocular and visual problems,5 and vascular occlusive disease.6 Occurrence of neurologie and psychiatrie abnormalities during the course of temporal arteritis has been weil recorded,?-9 but a limited number of cases have been reported in which the patient presented primarily with such a disturbance. 10· 11 When discussing neuropsychiatrie aspects, most recent comprehensive reviews1-3 of temporal arteritis refer to several articles describing patients who presented with sorne of the more typical manifestations as weil. Keith N. Shenberger John G. Meharg, Jr C. Darntll Lane Dr Shenberger was senior resident in internai medicine, Reading Hospital and Medical Center, West Reading, Pennsylvania. He is nowa fellow in connecli'te tissue diseases, DartmouthHitchcock Medical Center, Hanover, New Hampshire. Dr Meharg is in private practice and in the section of internai medicine, Reading Hospital and Medical Center. Dr Lane is in the department of pathology, Reading Hospital and Medical Center. Most often cited as a source on neuropsychiatrie aspects is a report of one case with depression (initial symptoms were. in fact. headaches l and a brief review of the literature with a compilation of neuropsychiatrie findings. 10 To our knowledge (which includes a MEDIARS search), temporal arteritis presenting as isolated ataxia with dementia has not been reported previously. We feel our case demonstrates an unusual and heretofore unreported presentation because of the atypical signs and symptoms as weil as the lack of classic findings. On the basis of this case, we wish to add temporal arteritis to the list of differentiai diagnoses for either ataxia or dementia in patients over age 50. We were impressed with the rather acute onset of ataxia and dementia in our patient at a time when her general condition was improving. Symptoms of unsteadiness and disorientation are vecy common in elderly patients when they have a systemic illness, are hospitalized, or are restricted to bed rest In addition, elevated ESR and mild normochromic normocytic anemia are often seen in such a clinical situation. Consequently, these findings often are not extensively investigated. In our case, only the remarkable acute TEMPORAL ARTERITIS•VOL 69/NO 2/FEBRUARY 1981/POSTGRADUATE MEDICINE Downloaded by [Australian Catholic University] at 16:34 10 August 2017 onset of ataxia and dementia caused us to probe further. The patienfs rapid response to glueocortieoid therapy suggests that vasculitis was the underlying cause of the neurologie symptoms and signs. In addition, angiographie studies and autopsies have clearly revealed cerebral angiitis in sorne patients with temporal arteritis. 12· 13 It is interesting to speculate on the relationship in our patient of temporal arteritis and hypoaetive deep tendon reflexes, transient renal failure, and abnormalliver funetion studies, as weil as elevated spinal fluid protein value. Ail of these have been reported in other cases.' 4 -16 Indeed, it is eonceivable that the carotid bruit, ehest pain, and abnormallung scan reported here were ail related to underlying vasculitis, sinœ sorne of these findings also have been reported in temporal arteritisP The patienfs elevated ESR and normochromie normocytie anemia were typical of temporal arteritis, but she did not have symptoms or local findings typical of temporal artery inflammation. Conclusion 1be case reported here illustrates some of the protean manifestations of temporal arterltis. Perhaps, as more cases with atypical manifestations are descrlbed, physieians will become more alert to the possibility of tiUs diag:nosis. It is now our polley to include temporal arterltis high on the list of differentiai diagnoses for any neuropsychiatrie, visual, or systemie complaint in an elderly patient, even in the absence of typical manifestations. A temporal artexy biopsy done relatively early in undiag:nosed illness References 1. Fauchald P, Rygvold 0, Oystese B. Temporal arterltls and polymyalgia rheumatlca: clinical and biopsy findings. Ann Intem Med 1972:77:845-52 2. Hamilton CR Jr, Shelley WM, TumultyPA. Giant cell arterttls: including temporal arterltls and polymyalgia rheumatlca. Medicine 1971 :50:1-27 3. Hamrln B. Polymyalgia arterttica. Acta Med Scand (Suppl) 1972:533:1-131 4. Hunder GG, Allen GL. The relatlonship between polymyalgia rheumatica and temporal arterttls. Gerlatrtcs 1973;28: 134-42 5. Simmons RJ, Cogan DG. Occult temporal arterltls. Arch Ophthal (Chic) 1962;68:38-48 6. Hunder GG, Ward LE, Burbank MK. Giant-cell arterltls producing an aortlc arch syndrome. Ann Intem Med 1967;66:578-82 7. Warrell DA. Godfrey S, Olsen EG. Giant-œll arterttis wlth perlpheral neuropathy. Lancet 1968;1:1010-3 8. Hart Cf. Formed vlsual hallucinations: a symptom of cranlal arterltls. Br Med J 1967;3:643-4 9. Hollenhorst RW, Brown JR, Wagener HP, et al Neurologie aspects of VOL ~9/NO 2/FEBRUARY 1981/POSTGRADUATE MEDICINE•TEMPORAL ARTERITIS may revea1 a vexy treatable cause. We wish to emphasize the need for early consideration of temporal arterltis in the elderly patient with an elevated ESR and any unexplained neuropsychiatrie problem. RJIII Mrs Sharon Walters provlded technical assistance in the preparation of this report. Address reprtnt requests to John G. Meharg. Jr. MD. 39 E Lancaster Ave. Shillington. PA 19607. temporal arterttis. Neurology 1960:10: 490-8 10. Vereker R. The psychiatrie aspects of temporal arterttis. J Ment Sei 1952: 98:280-6 11. PaulleyJW, HughesJP. Giant-cell arterltis. or arterltis of the aged. Br Med J 1960;2:1562-7 12. CochranJW, FoxJH, KellyMP. Reversible mental symptoms in temporal arterltis. J Nerv Ment Dis 1978;166:446-7 13. Enzmann D, ScottWR. Intracranial involvement of giant-cell arterltis. Neurology 1977:27:794-7 14. lfinck VC, Carter CC, Rippey JG. Giant cell (cranial) arterttls: a case wlth angiographie abnormalities. Am J Roentgen 1964;92:769-75 15. Balmforth GV. Temporal arterltis and renal fallure. Arch Intem Med 1964;113:230-4 16. Dickson ER, Maldonado JE, Sheps SG, et al Systernic giant-cell arterltis wlth polymyalgia rheumatica. reversible abnormalities of liver functlon. JAMA 1973;224:1496-8 17. Cooke wr, Cloake PC, GovanAD, et al. Temporal arterttis: a generalized vascular disease. Q J Med 1946;15:47-75 249