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An Autopsy Case of Elderly Onset Brainstem Acute Disseminated Encephalomyelitis
Yasuyuki Takai1,2, Shinsuke Tobisawa1, Asuka Funai1
1Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
Abstract:
Acute disseminated encephalomyelitis (ADEM), which is a disease that causes multifocal inflammatory demyelination of the central nervous system, occurs predominantly in children and young adults. We report an autopsy case of an elderly man with brainstem ADEM that progressed over a period of about 3 months. An 82-year-old man developed disturbance of consciousness, dysphagia, and ataxic gait over a period of about 3 months. He was admitted to another hospital for aspiration pneumonia and recovered but was transferred to our hospital due to prolonged disturbance of consciousness. The patient was able to follow simple commands but had a tendency to somnolence. In addition to meningeal stimulation signs, the patient had left-dominant upper and lower limb ataxia and right-dominant limb spasticity. Brain FLAIR/T2-weighted imaging showed high-intensity lesions from the brainstem to the middle cerebellar peduncle bilaterally, medulla oblongata and upper cervical spinal cord, and T1-weighted imaging revealed contrast-enhanced lesions in the left middle cerebellar peduncle and cervical spinal cord. Although spinal fluid examination revealed elevated proteins, other laboratory tests indicated no evidence of infection, vasculitis, collagen diseases or tumors, and anti-ganglioside, anti-AQP4 and anti-MOG antibodies were negative. After admission, the patient again developed aspiration pneumonia, which progressed to acute respiratory distress syndrome, and he died on the 15th day of hospitalization. Autopsy findings indicated acute and subacute demyelination mainly in the brainstem and cerebellum, and perivascular lymphocyte and macrophage infiltration in the areas of demyelination. A postmortem diagnosis of ADEM was made based on the generally monophasic course of the disease and the absence of regenerating myelinated sheaths. There are very few reports of elderly patients with brainstem ADEM. ADEM should be considered as a differential diagnosis in patients with brainstem encephalitis.
Insights
This case report details an elderly man with brainstem acute disseminated encephalomyelitis (ADEM), a rare presentation of this central nervous system inflammatory disease. Autopsy confirmed ADEM, highlighting its potential in older adults with brainstem encephalitis symptoms.
Area of Science:
- Neurology
- Pathology
Background:
- Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system, typically affecting children and young adults.
- Brainstem involvement in ADEM is uncommon, especially in the elderly population.
Observation:
- An 82-year-old male presented with a 3-month history of progressive neurological decline, including altered consciousness, dysphagia, and ataxia.
- Clinical and imaging findings revealed extensive demyelination in the brainstem and spinal cord, with negative infectious, autoimmune, and neoplastic workups.
- The patient experienced recurrent aspiration pneumonia, leading to acute respiratory distress syndrome and death.
Findings:
- Autopsy confirmed acute and subacute demyelination primarily in the brainstem and cerebellum, with associated perivascular inflammatory cell infiltration.
- The pathological findings and monophasic disease course supported a diagnosis of ADEM.
- Key antibodies associated with other demyelinating diseases (anti-ganglioside, anti-AQP4, anti-MOG) were negative.
Implications:
- This case underscores the importance of considering ADEM in the differential diagnosis of elderly patients presenting with brainstem encephalitis.
- The rarity of ADEM in the elderly highlights the need for increased awareness and further research into atypical presentations.
- Early recognition and diagnosis of ADEM, even in older individuals, are crucial for appropriate management and understanding of neurological disorders.
