Miliary brain metastases presenting as rapidly progressive dementia
Eloy Rivas1, Joaquin Sanchez-Herrero, Miguel Alonso
1Department of Pathology and Neuropathology, Meixoeiro Hospital, Vigo, Spain.
Abstract:
We report the case of a 79-year-old woman who developed a rapidly progressive dementia (RPD) with severe memory impairment, early visual hallucinations and extrapyramidal signs. Symptoms started suddenly after hip replacement surgery following an accidental fall. Motor epileptic seizures appeared at the end of the illness. Dementia worsened gradually leading to akinetic mutism. She died five and a half months after the onset of symptoms. MRI showed cerebral atrophy but failed to detect any other lesion. Results of all laboratory tests performed were negative. After the most frequent treatable diseases were excluded, the diagnosis of dementia with Lewy bodies was initially considered. CJD was also suggested based on the rapid evolution of the disease and the positivity of 14-3-3 protein in CSF. Neuropathological examination revealed an extensive miliary metastatic dissemination from an unknown primary adenocarcinoma. Pulmonary origin was suggested according to the immunohistochemical profile. Histopathological changes of Alzheimer's disease were also observed in the cerebral cortex and hippocampus. Neither Lewy bodies nor PrP deposits were found. The sudden onset of the dementia just after the hip replacement surgery raises the possibility of a pathological fracture with secondary tumoral microembolic dissemination. Despite its rarity, this entity should be included in the differential diagnosis of RPD. This case illustrates the definite importance of neuropathological post-mortem examination in order to elucidate the different types of dementia.
Insights
A rare case of rapidly progressive dementia (RPD) was caused by widespread cancer metastasis, not typical neurodegenerative diseases. This highlights the importance of considering cancer in RPD differential diagnoses.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Rapidly progressive dementia (RPD) presents a diagnostic challenge, often mimicking neurodegenerative conditions like dementia with Lewy bodies or Creutzfeldt-Jakob disease (CJD).
- The sudden onset of neurological symptoms post-surgery necessitates a broad differential diagnosis, including rare causes.
- Metastatic cancer can present with diverse neurological symptoms, including cognitive decline.
Observation:
- A 79-year-old woman developed RPD with severe memory loss, visual hallucinations, and extrapyramidal signs after hip surgery.
- Neurological deterioration progressed to akinetic mutism over five months, with seizures appearing terminally.
- Initial investigations, including MRI and CSF analysis, were inconclusive for common causes of RPD.
Findings:
- Neuropathological examination revealed extensive miliary metastatic adenocarcinoma, likely of pulmonary origin, despite no known primary tumor.
- Alzheimer's disease histopathological changes were noted, but Lewy bodies and PrP deposits were absent.
- The clinical presentation suggested a possible link between a pathological fracture, microembolic dissemination, and the sudden onset of dementia.
Implications:
- Metastatic cancer, particularly from an unknown primary, should be considered in the differential diagnosis of RPD, especially with sudden onset post-trauma or surgery.
- This case underscores the critical role of post-mortem neuropathological examination in identifying rare causes of dementia.
- Understanding such rare presentations can improve diagnostic accuracy and potentially guide future therapeutic strategies for dementia.
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