Rapidly progressing dementia: Difference between revisions
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m (Text replacement - "Creutzfeld-Jakob disease" to "Creutzfeldt-Jakob disease") |
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== Background == |
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* Subacute and accelerated decline, developing over 2 years |
* Subacute and accelerated decline, developing over 2 years |
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* Must rule out Creutzfeldt-Jakob disease (CJD) |
* Must rule out [[Creutzfeldt-Jakob disease]] (CJD) |
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== Differential Diagnosis == |
== Differential Diagnosis == |
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* Creutzfeldt-Jakob disease (CJD) |
* [[Creutzfeldt-Jakob disease]] (CJD) |
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* 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 |
** [[Frontotemporal dementia]], [[progressive supranuclear palsy]], [[Lewy body dementia]], and [[Alzheimer dementia]] |
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* Limbic |
* [[Limbic encephalitis]]: short-term memory loss, executive dysfuntion, personality changes, panic attacks, and delusions and hallucinations, as well as seizures |
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** Paraneoplastic syndromes |
** Paraneoplastic syndromes |
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*** Anti-Hu: often found in small cell lung cancer |
*** Anti-Hu: often found in small cell lung cancer |
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*** VGKC |
*** VGKC |
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** 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 |
** [[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 |
* [[Hashimoto encephalitis]] (steroid-responsive encephalopathy): often in patients with other autoimmune disorders, diagnosed with anti-TPO and anti-TG antibodies |
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* Vascular |
* Vascular |
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** Stroke, vascular dementia |
** [[Stroke]], [[vascular dementia]] |
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** CADASIL |
** [[CADASIL]] |
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** Thrombotic thrombocytopenic purpura (TTP) |
** [[Thrombotic thrombocytopenic purpura]] (TTP) |
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** Hyperviscosity |
** Hyperviscosity |
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** Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies) |
** Syndromes/paraproteinemias ([[polycythemia]], [[Monocloncal gammopathy|monocloncal gammopathies]]) |
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** Hypoxic-ischemic encephalopathy |
** [[Hypoxic-ischemic encephalopathy]] |
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* Infectious |
* Infectious |
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** Whipple disease |
** [[Whipple disease]] |
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** Syphilis |
** [[Syphilis]] |
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** Lyme disease |
** [[Lyme disease]] |
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** Subacute sclerosing |
** [[Subacute sclerosing panencephalitis]] (SSPE) (measles) |
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** HIV-associated dementia |
** [[HIV-associated dementia]] |
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** Progressive multifocal leukoencephalopathy (JC virus) |
** [[Progressive multifocal leukoencephalopathy]] (JC virus) |
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* Toxic/metabolic |
* Toxic/metabolic |
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** Vitamin B12 deficiency |
** [[Vitamin B12 deficiency]] |
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** Thiamine deficiency |
** [[Thiamine deficiency]] |
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** Niacin deficiency |
** [[Niacin deficiency]] |
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** Folate deficiency |
** [[Folate deficiency]] |
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** Uremic encephalopathy |
** [[Uremic encephalopathy]] |
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** Wilson disease |
** [[Wilson disease]] |
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** Hepatic encephalopathy |
** [[Hepatic encephalopathy]] |
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** Porphyria |
** [[Porphyria]] |
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* Heavy |
* [[Heavy metal toxicity]] |
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** Bismuth toxicity |
** [[Bismuth toxicity]] |
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** Alcohol toxicity |
** [[Alcohol toxicity]] |
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** Lithium toxicity |
** [[Lithium toxicity]] |
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** Mercury toxicity |
** [[Mercury toxicity]] |
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** Arsenic toxicity |
** [[Arsenic toxicity]] |
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** Lead |
** [[Lead toxicity]] |
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* Electrolyte abnormalities |
* Electrolyte abnormalities |
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** Kuf disease |
** [[Kuf disease]] |
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** Methylmalonic acidemia |
** [[Methylmalonic acidemia]] |
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** Mitochondrial encephalopathies |
** [[Mitochondrial encephalopathy|Mitochondrial encephalopathies]] |
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* Autoimmune |
* Autoimmune |
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** Paraneoplastic limbic encephalitis |
** [[Paraneoplastic limbic encephalitis]] |
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** Anti-VGKC-E |
** Anti-VGKC-E |
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** Hashimoto encephalopathy |
** [[Hashimoto encephalopathy]] |
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** Lupus cerebritis |
** [[Lupus cerebritis]] |
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** [[Neurosarcoidosis]] |
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** Sarcoid |
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** CNS vasculitis |
** [[CNS vasculitis]] |
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** Celiac disease |
** [[Celiac disease]] |
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* Metastases/neoplastic |
* Metastases/neoplastic |
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** CNS metastases |
** CNS metastases |
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** Primary CNS lymphoma |
** [[Primary CNS lymphoma]] |
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** Intravascular lymphoma |
** Intravascular lymphoma |
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** Lymphomatoid granulomatosis |
** [[Lymphomatoid granulomatosis]] |
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** Gliomatosis cerebri |
** [[Gliomatosis cerebri]] |
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* Iatrogenic/idiopathic |
* Iatrogenic/idiopathic |
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** Central pontine myelinolysis |
** [[Central pontine myelinolysis]] |
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** Insulin-induced hypoglycemia |
** Insulin-induced [[hypoglycemia]] |
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** Normal pressure hydrocephalus (NPH) |
** [[Normal pressure hydrocephalus]] (NPH) |
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* Neurodegenerative |
* Neurodegenerative |
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** |
** [[Alzheimer dementia]] |
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** Creutzfeldt-Jakob disease |
** [[Creutzfeldt-Jakob disease]] |
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* Frontotemporal syndrome |
* Frontotemporal syndrome |
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** Behavioural variant frontotemporal dementia |
** Behavioural variant [[frontotemporal dementia]] |
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** Semantic dementia |
** Semantic dementia |
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** Progressive non-fluent aphasia |
** Progressive non-fluent aphasia |
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** Frontotemporal dementia-motor neuron disease |
** Frontotemporal dementia-motor neuron disease |
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* Parkinson syndrome |
* [[Parkinson syndrome]] |
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** Dementia with |
** [[Dementia with Lewy bodies]] |
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** Corticobasal degeneration |
** [[Corticobasal degeneration]] |
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** Progressive supranuclear palsy |
** [[Progressive supranuclear palsy]] |
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** Parkinson disease dementia |
** [[Parkinson disease dementia]] |
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** High definition |
** High definition |
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* Systemic |
* Systemic |
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** Vasculitis (giant cell arteritis) |
** [[Vasculitis]] ([[giant cell arteritis]]) |
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** Sleep apnea |
** [[Sleep apnea]] |
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[[Category:Neurology]] |
[[Category:Neurology]] |
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Revision as of 16:19, 20 August 2021
Background
- 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
- 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
