Rapidly progressing dementia
From IDWiki
Background
- Subacute and accelerated decline, developing over 2 years
- Must rule out Creutzfeldt-Jakob disease (CJD)
Differential Diagnosis
- Creutzfeldt-Jakob disease (CJD), especially when myoclonus is present
- Atypical course of a more common form of dementia
- Limbic encephalitis: short-term memory loss, executive dysfuntion, personality changes, panic attacks, and delusions and hallucinations, as well as seizures
- Paraneoplastic syndromes
- Anti-Hu: often found in small cell lung cancer
- CV2: only found in small cell lung cancer or thymoma
- Ma2: testicular germ-cell tumours, breast cancer, and NSCLC
- VGKC
- Anti-voltage-gated potassium channel encephalopathy (VGKC-E): often has hyponatremia
- HSV encephalitis
- Paraneoplastic syndromes
- Hashimoto encephalitis (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies
Extended Differential Diagnosis
- Vascular
- Stroke, vascular dementia
- CADASIL
- Thrombotic thrombocytopenic purpura (TTP)
- Hyperviscosity
- Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies)
- Hypoxic-ischemic encephalopathy
- Infectious
- Toxic/metabolic
- Heavy metal toxicity
- Electrolyte abnormalities
- Autoimmune
- Metastases/neoplastic
- CNS metastases
- Primary CNS lymphoma
- Intravascular lymphoma
- Lymphomatoid granulomatosis
- Gliomatosis cerebri
- Iatrogenic/idiopathic
- Central pontine myelinolysis
- Insulin-induced hypoglycemia
- Normal pressure hydrocephalus (NPH)
- Neurodegenerative
- Frontotemporal syndrome
- Behavioural variant frontotemporal dementia
- Semantic dementia
- Progressive non-fluent aphasia
- Frontotemporal dementia-motor neuron disease
- Parkinson syndrome
- Systemic
Investigations
- MRI: CJD is the only degenerative condition that causes hyperintensities in DWI and FLAIR
