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
* Must rule out Creutzfeldt-Jakob disease (CJD)
* Must rule out [[Creutzfeldt-Jakob disease]] (CJD)


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


* Creutzfeldt-Jakob disease (CJD)
* [[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
** Frontotemporal dementia, progressive supranuclear palsy, Lewy Body dementia, and Alzheimer 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
* [[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
** HSV encephalitis
** [[HSV encephalitis]]
* Hashimoto encephalitis (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies
* [[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
** Stroke, vascular dementia
** [[Stroke]], [[vascular dementia]]
** CADASIL
** [[CADASIL]]
** Thrombotic thrombocytopenic purpura (TTP)
** [[Thrombotic thrombocytopenic purpura]] (TTP)
** Hyperviscosity
** Hyperviscosity
** Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies)
** Syndromes/paraproteinemias ([[polycythemia]], [[Monocloncal gammopathy|monocloncal gammopathies]])
** Hypoxic-ischemic encephalopathy
** [[Hypoxic-ischemic encephalopathy]]
* Infectious
* Infectious
** Whipple disease
** [[Whipple disease]]
** Syphilis
** [[Syphilis]]
** Lyme disease
** [[Lyme disease]]
** Subacute sclerosing panencphalitis (SSPE) (measles)
** [[Subacute sclerosing panencephalitis]] (SSPE) (measles)
** HIV-associated dementia
** [[HIV-associated dementia]]
** Progressive multifocal leukoencephalopathy (JC virus)
** [[Progressive multifocal leukoencephalopathy]] (JC virus)
* Toxic/metabolic
* Toxic/metabolic
** Vitamin B12 deficiency
** [[Vitamin B12 deficiency]]
** Thiamine deficiency
** [[Thiamine deficiency]]
** Niacin deficiency
** [[Niacin deficiency]]
** Folate deficiency
** [[Folate deficiency]]
** Uremic encephalopathy
** [[Uremic encephalopathy]]
** Wilson disease
** [[Wilson disease]]
** Hepatic encephalopathy
** [[Hepatic encephalopathy]]
** Porphyria
** [[Porphyria]]
* Heavy metals
* [[Heavy metal toxicity]]
** Bismuth toxicity
** [[Bismuth toxicity]]
** Alcohol toxicity
** [[Alcohol toxicity]]
** Lithium toxicity
** [[Lithium toxicity]]
** Mercury toxicity
** [[Mercury toxicity]]
** Arsenic toxicity
** [[Arsenic toxicity]]
** Lead
** [[Lead toxicity]]
* Electrolyte abnormalities
* Electrolyte abnormalities
** Kuf disease
** [[Kuf disease]]
** Methylmalonic acidemia
** [[Methylmalonic acidemia]]
** Mitochondrial encephalopathies
** [[Mitochondrial encephalopathy|Mitochondrial encephalopathies]]
* Autoimmune
* Autoimmune
** Paraneoplastic limbic encephalitis
** [[Paraneoplastic limbic encephalitis]]
** Anti-VGKC-E
** Anti-VGKC-E
** Hashimoto encephalopathy
** [[Hashimoto encephalopathy]]
** Lupus cerebritis
** [[Lupus cerebritis]]
** [[Neurosarcoidosis]]
** Sarcoid
** CNS vasculitis
** [[CNS vasculitis]]
** Celiac disease
** [[Celiac disease]]
* Metastases/neoplastic
* Metastases/neoplastic
** CNS metastases
** CNS metastases
** Primary CNS lymphoma
** [[Primary CNS lymphoma]]
** Intravascular lymphoma
** Intravascular lymphoma
** Lymphomatoid granulomatosis
** [[Lymphomatoid granulomatosis]]
** Gliomatosis cerebri
** [[Gliomatosis cerebri]]
* Iatrogenic/idiopathic
* Iatrogenic/idiopathic
** Central pontine myelinolysis
** [[Central pontine myelinolysis]]
** Insulin-induced hypoglycemia
** Insulin-induced [[hypoglycemia]]
** Normal pressure hydrocephalus (NPH)
** [[Normal pressure hydrocephalus]] (NPH)
* Neurodegenerative
* Neurodegenerative
** Alzheumer dementia
** [[Alzheimer dementia]]
** Creutzfeldt-Jakob disease
** [[Creutzfeldt-Jakob disease]]
* Frontotemporal syndrome
* Frontotemporal syndrome
** Behavioural variant frontotemporal dementia
** 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
* Parkinson syndrome
* [[Parkinson syndrome]]
** Dementia with lewy bodies
** [[Dementia with Lewy bodies]]
** Corticobasal degeneration
** [[Corticobasal degeneration]]
** Progressive supranuclear palsy
** [[Progressive supranuclear palsy]]
** Parkinson disease dementia
** [[Parkinson disease dementia]]
** High definition
** High definition
* Systemic
* Systemic
** Vasculitis (giant cell arteritis)
** [[Vasculitis]] ([[giant cell arteritis]])
** 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 18:09, 15 July 2022

Background

Differential Diagnosis

Extended Differential Diagnosis

Investigations

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