Rapidly progressing dementia: Difference between revisions

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== Background ==
 
* Subacute and accelerated decline, developing over 2 years
 
* Subacute and accelerated decline, developing over 2 years
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* Must rule out Creutzfeld-Jakob disease (CJD)
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* Must rule out [[Creutzfeldt-Jakob disease]] (CJD)
   
 
== Differential Diagnosis ==
 
== Differential Diagnosis ==
   
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* Creutzfeld-Jakob disease (CJD)
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* [[Creutzfeldt-Jakob disease]] (CJD), especially when myoclonus is present
 
* Atypical course of a more common form of dementia
 
* Atypical course of a more common form of dementia
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** Frontotemporal dementia, progressive supranuclear palsy, Lewy Body dementia, and Alzheimer dementia
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** [[Frontotemporal dementia]], [[progressive supranuclear palsy]], [[Lewy body dementia]], and [[Alzheimer dementia]]
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* Limbic encphalitis: short-term memory loss, executive dysfuntion, personality changes, panic attacks, and delusions and hallucinations, as well as seizures
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* [[Limbic encephalitis]]: short-term memory loss, executive dysfuntion, personality changes, panic attacks, and delusions and hallucinations, as well as seizures
 
** Paraneoplastic syndromes
 
** Paraneoplastic syndromes
 
*** Anti-Hu: often found in small cell lung cancer
 
*** Anti-Hu: often found in small cell lung cancer
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*** VGKC
 
*** VGKC
 
** Anti-voltage-gated potassium channel encephalopathy (VGKC-E): often has hyponatremia
 
** Anti-voltage-gated potassium channel encephalopathy (VGKC-E): often has hyponatremia
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** HSV encephalitis
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** [[HSV encephalitis]]
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* Hashimoto encephalitis (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies
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* [[Hashimoto encephalitis]] (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies
   
 
=== Extended Differential Diagnosis ===
== Investigations ==
 
−  
* MRI: CJD is the only degenerative condition that causes hyperintensities in DWI and FLAIR
 
−  
== Extended Differential Diagnosis ==
 
   
 
* Vascular
 
* Vascular
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** Stroke, vascular dementia
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** [[Stroke]], [[vascular dementia]]
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** CADASIL
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** [[CADASIL]]
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** Thrombotic thrombocytopenic purpura (TTP)
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** [[Thrombotic thrombocytopenic purpura]] (TTP)
 
** Hyperviscosity
 
** Hyperviscosity
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** Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies)
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** Syndromes/paraproteinemias ([[polycythemia]], [[Monocloncal gammopathy|monocloncal gammopathies]])
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** Hypoxic-ischemic encephalopathy
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** [[Hypoxic-ischemic encephalopathy]]
 
* Infectious
 
* Infectious
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** Whipple disease
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** [[Whipple disease]]
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** Syphilis
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** [[Syphilis]]
−
** Lyme disease
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** [[Lyme disease]]
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** Subacute sclerosing panencphalitis (SSPE) (measles)
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** [[Subacute sclerosing panencephalitis]] (SSPE) (measles)
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** HIV-associated dementia
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** [[HIV-associated dementia]]
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** Progressive multifocal leukoencephalopathy (JC virus)
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** [[Progressive multifocal leukoencephalopathy]] (JC virus)
 
* Toxic/metabolic
 
* Toxic/metabolic
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** Vitamin B12 deficiency
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** [[Vitamin B12 deficiency]]
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** Thiamine deficiency
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** [[Thiamine deficiency]]
−
** Niacin deficiency
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** [[Niacin deficiency]]
−
** Folate deficiency
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** [[Folate deficiency]]
−
** Uremic encephalopathy
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** [[Uremic encephalopathy]]
−
** Wilson disease
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** [[Wilson disease]]
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** Hepatic encephalopathy
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** [[Hepatic encephalopathy]]
−
** Porphyria
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** [[Porphyria]]
−
* Heavy metals
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* [[Heavy metal toxicity]]
−
** Bismuth toxicity
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** [[Bismuth toxicity]]
−
** Alcohol toxicity
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** [[Alcohol toxicity]]
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** Lithium toxicity
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** [[Lithium toxicity]]
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** Mercury toxicity
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** [[Mercury toxicity]]
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** Arsenic toxicity
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** [[Arsenic toxicity]]
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** Lead
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** [[Lead toxicity]]
 
* Electrolyte abnormalities
 
* Electrolyte abnormalities
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** Kuf disease
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** [[Kuf disease]]
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** Methylmalonic acidemia
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** [[Methylmalonic acidemia]]
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** Mitochondrial encephalopathies
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** [[Mitochondrial encephalopathy|Mitochondrial encephalopathies]]
 
* Autoimmune
 
* Autoimmune
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** Paraneoplastic limbic encephalitis
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** [[Paraneoplastic limbic encephalitis]]
 
** Anti-VGKC-E
 
** Anti-VGKC-E
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** Hashimoto encephalopathy
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** [[Hashimoto encephalopathy]]
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** Lupus cerebritis
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** [[Lupus cerebritis]]
  +
** [[Neurosarcoidosis]]
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** Sarcoid
 
−
** CNS vasculitis
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** [[CNS vasculitis]]
−
** Celiac disease
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** [[Celiac disease]]
 
* Metastases/neoplastic
 
* Metastases/neoplastic
 
** CNS metastases
 
** CNS metastases
−
** Primary CNS lymphoma
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** [[Primary CNS lymphoma]]
 
** Intravascular lymphoma
 
** Intravascular lymphoma
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** Lymphomatoid granulomatosis
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** [[Lymphomatoid granulomatosis]]
−
** Gliomatosis cerebri
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** [[Gliomatosis cerebri]]
 
* Iatrogenic/idiopathic
 
* Iatrogenic/idiopathic
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** Central pontine myelinolysis
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** [[Central pontine myelinolysis]]
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** Insulin-induced hypoglycemia
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** Insulin-induced [[hypoglycemia]]
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** Normal pressure hydrocephalus (NPH)
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** [[Normal pressure hydrocephalus]] (NPH)
 
* Neurodegenerative
 
* Neurodegenerative
−
** Alzheumer dementia
+
** [[Alzheimer dementia]]
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** Creutzfeld-Jakob disease
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** [[Creutzfeldt-Jakob disease]]
 
* Frontotemporal syndrome
 
* Frontotemporal syndrome
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** Behavioural variant frontotemporal dementia
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** Behavioural variant [[frontotemporal dementia]]
 
** Semantic dementia
 
** Semantic dementia
 
** Progressive non-fluent aphasia
 
** Progressive non-fluent aphasia
 
** Frontotemporal dementia-motor neuron disease
 
** Frontotemporal dementia-motor neuron disease
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* Parkinson syndrome
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* [[Parkinson syndrome]]
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** Dementia with lewy bodies
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** [[Dementia with Lewy bodies]]
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** Corticobasal degeneration
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** [[Corticobasal degeneration]]
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** Progressive supranuclear palsy
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** [[Progressive supranuclear palsy]]
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** Parkinson disease dementia
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** [[Parkinson disease dementia]]
 
** High definition
 
** High definition
 
* Systemic
 
* Systemic
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** Vasculitis (giant cell arteritis)
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** [[Vasculitis]] ([[giant cell arteritis]])
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** Sleep apnea
+
** [[Sleep apnea]]
  +
 
== Investigations ==
 
* MRI: CJD is the only degenerative condition that causes hyperintensities in DWI and FLAIR
   
 
[[Category:Neurology]]
 
[[Category:Neurology]]

Latest revision as of 14:09, 15 July 2022

Background

Differential Diagnosis

Extended Differential Diagnosis

Investigations

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