Rapidly progressing dementia: Difference between revisions

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m (Text replacement - "Creutzfeld-Jakob disease" to "Creutzfeldt-Jakob disease")
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* Subacute and accelerated decline, developing over 2 years
* Subacute and accelerated decline, developing over 2 years
* Must rule out Creutzfeld-Jakob disease (CJD)
* Must rule out Creutzfeldt-Jakob disease (CJD)


== Differential Diagnosis ==
== Differential Diagnosis ==


* Creutzfeld-Jakob disease (CJD)
* Creutzfeldt-Jakob disease (CJD)
* Atypical course of a more common form of dementia
* Atypical course of a more common form of dementia
** Frontotemporal dementia, progressive supranuclear palsy, Lewy Body dementia, and Alzheimer dementia
** Frontotemporal dementia, progressive supranuclear palsy, Lewy Body dementia, and Alzheimer dementia
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* Neurodegenerative
* Neurodegenerative
** Alzheumer dementia
** Alzheumer dementia
** Creutzfeld-Jakob disease
** Creutzfeldt-Jakob disease
* Frontotemporal syndrome
* Frontotemporal syndrome
** Behavioural variant frontotemporal dementia
** Behavioural variant frontotemporal dementia

Revision as of 15:32, 5 November 2020

  • Subacute and accelerated decline, developing over 2 years
  • Must rule out Creutzfeldt-Jakob disease (CJD)

Differential Diagnosis

  • Creutzfeldt-Jakob disease (CJD)
  • Atypical course of a more common form of dementia
    • Frontotemporal dementia, progressive supranuclear palsy, Lewy Body dementia, and Alzheimer dementia
  • Limbic encphalitis: 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
  • Hashimoto encephalitis (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies

Investigations

  • MRI: CJD is the only degenerative condition that causes hyperintensities in DWI and FLAIR

Extended Differential Diagnosis

  • Vascular
    • Stroke, vascular dementia
    • CADASIL
    • Thrombotic thrombocytopenic purpura (TTP)
    • Hyperviscosity
    • Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies)
    • Hypoxic-ischemic encephalopathy
  • Infectious
    • Whipple disease
    • Syphilis
    • Lyme disease
    • Subacute sclerosing panencphalitis (SSPE) (measles)
    • HIV-associated dementia
    • Progressive multifocal leukoencephalopathy (JC virus)
  • Toxic/metabolic
    • Vitamin B12 deficiency
    • Thiamine deficiency
    • Niacin deficiency
    • Folate deficiency
    • Uremic encephalopathy
    • Wilson disease
    • Hepatic encephalopathy
    • Porphyria
  • Heavy metals
    • Bismuth toxicity
    • Alcohol toxicity
    • Lithium toxicity
    • Mercury toxicity
    • Arsenic toxicity
    • Lead
  • Electrolyte abnormalities
    • Kuf disease
    • Methylmalonic acidemia
    • Mitochondrial encephalopathies
  • Autoimmune
    • Paraneoplastic limbic encephalitis
    • Anti-VGKC-E
    • Hashimoto encephalopathy
    • Lupus cerebritis
    • Sarcoid
    • CNS vasculitis
    • Celiac disease
  • 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
    • Alzheumer dementia
    • Creutzfeldt-Jakob disease
  • Frontotemporal syndrome
    • Behavioural variant frontotemporal dementia
    • Semantic dementia
    • Progressive non-fluent aphasia
    • Frontotemporal dementia-motor neuron disease
  • Parkinson syndrome
    • Dementia with lewy bodies
    • Corticobasal degeneration
    • Progressive supranuclear palsy
    • Parkinson disease dementia
    • High definition
  • Systemic
    • Vasculitis (giant cell arteritis)
    • Sleep apnea