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Título

CREUTZFELDT-JAKOB DISEASE HEIDENHAIN SUBTYPE: CASE REPORT

RESUMO

CASE REPORT
J.T.M., female, 67, alcoholic. Social isolation begins and evolves with a bilateral visual deficit, abdominal pain, and headache. She presented with dental septicemia. At examination: miotic pupils, photoreactive pupils, upper limbs with spasticity, paratonia, and myoclonus. She didn't interact and didn't obey commands. In addition, patellar hyperreflexia and bilateral flexion on plantar reflex. Evolution with gait ataxia, changes in motor coordination, ideomotor and speech apraxia, and behavioral changes were present. During hospitalization, she evolved with bilateral extension at plantar reflex. 14.3.3 protein was dosed due to the hypothesis of spongiform encephalopathy. Its result showed a value of 33,712 (0-19,999). There has been an intense investigation of differential diagnoses. The patient began to have anticipated complications and progressed to support.

DISCUSSION
Regarding CJD, about 80 to 85% of cases are caused by the sporadic form, with prevalence in the elderly population, between the 5th and 6th decade of life, with no sex differentiation and absence of distinct transmission patterns. In Heidenhain subtype CJD, the Parieto-Occipital cortex is affected, affecting the primary visual area and causing image changes such as cortical hyperintensities on T2/FLAIR MRI and basal ganglia (striatum and thalamus). The electroencephalography (EEG) shows periodic, three-phase acute waves, predominantly in the posterior areas. 14.3.3 protein can also be dosed from the cerebrospinal fluid. Prognosis is one year of life after the onset of symptoms. The classic presentation of this variant involves cortical blindness capable of generating various types of visual changes, visual field restriction, hallucinations, and visuospatial recognition. Neuropsychiatric symptoms culminated in agitation and aggression. The symptoms progressed in a short time (60 days) until presenting akinetic mutism, typical of the disease. Measurement of 14.3.3 protein in the CSF, among the presence of at least two supportive clinical criteria, and the association with progressive dementia were crucial for defining a probable diagnostic hypothesis.

FINAL COMMENTS
The signs and symptoms described in the literature were compatible with those presented in this case, representing the Heidenhain subtype of sCJD. There is no specific treatment. Currently, the basis of the therapeutic approach is on supportive measures.

Palavras Chave

Creutzfeldt-Jakob, Heidenhain, 14.3.3 protein, progressive dementia

Área

Neurologia Cognitiva E Do Envelhecimento