Clinical Characteristics of Chronic Creutzfeldt-Jakob Disease

Abstract

Chronic Creutzfeldt-Jakob disease, presenting in later life, may be difficult to distinguish from other dementing illnesses. Patients with this disease may lack the characteristic myoclonus and electroencephalographic complexes. We report four patients with a slowly progressive dementia who were misdiagnosed during life and had a spongiform encephalopathy at autopsy. All four patients had early extrapyramidal rigidity, primitive reflexes, and other neurologic signs. The presence of these neurologic findings early in the course of a dementia suggests that clinicians should consider chronic Creutzfeldt-Jakob disease, evaluate the patient with electroencephalographic studies, and emphasize universal tissue precautions.

Get full access to this article

View all access options for this article.

References

1. Brown P , Rodgers-Johnson P , Cathala F , et al. Creutzfeldt-Jakob disease of long duration: clinicopathological characteristics, transmissibility, and differential diagnosis. Ann Neurol 1984; 16:295–304.

2. Knight R. Creutzfeldt-Jakob disease. Br J Hosp Med 1989; 41:165–166, 168–171.

3. Mendez MF , Mastri AR , Sung JH , Frey WH II . Clinically diagnosed Alzheimer's disease: neuropathological findings in 650 cases. Alzheimer Dis Assoc Disord 1992; 6:35–43.

4. Boller F , Lopez OL , Moossy J. Diagnosis of dementia: clinicopathologic correlations. Neurology 1989; 39:76–79, 1559–1560.

5. Zochodne DW , Young GB , McLachlan RS , et al. Creutzfeldt-Jakob disease without periodic sharp wave complexes: a clinical, electroencephalographic, and pathologic study. Neurology 1988; 38:1056–1060.

6. Case records of the Massachusetts General Hospital. Case 49–1983. A 75-year-old man with progressive dementia. N Engl J Med 1983; 309:1440–1449.

7. Kovanen J , Erkinjuntti T , Iivanainen M , et al. Cerebral MR and CT imaging in Creutzfeldt-Jakob disease. J Comput Assist Tomogr 1985; 9:125–128.

8. Bendheim PE . The human spongiform encephalopathies. Neurol Clin 1984; 2:281–298.

9. Brown P , Cathala F , Castaigne P , Gajdusek DC . Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 1986; 20: 597–602.

10.

10. Masullo C , Pocchiari M , Neri G , et al. A retrospective study of Creutzfeldt-Jakob disease in Italy (1972-1986). Eur J Epidemiol 1988; 4:482–487.

11.

11. Malin JP , Weissenborn K , Haas J , Walter GF . Unusual EEG findings in a case of Creutzfeldt-Jakob disease. J Neurol 1987; 234:55–58.

12.

12. Erkulwater S , Pillai R. Amantadine and the end-stage dementia of Alzheimer's type. South Med J 1989; 82:550–554.

13.

13. Hauser WA , Morris ML , Heston LL , Anderson VE . Seizures and myoclonus in patients with Alzheimer's disease. Neurology 1986; 36:1226–1260.

14.

14. Rosenberg RN , White CL III , Brown P , et al. Precautions in handling tissues, fluids, and other contaminated materials from patients with documented or suspected Creutzfeldt-Jakob disease. Ann Neurol 1986; 19:75–77.