Rapidly progressing dementia: Difference between revisions
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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 |
+ | * Must rule out [[Creutzfeldt-Jakob disease]] (CJD) |
== Differential Diagnosis == |
== Differential Diagnosis == |
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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 |
+ | ** [[Frontotemporal dementia]], [[progressive supranuclear palsy]], [[Lewy body dementia]], and [[Alzheimer dementia]] |
− | * Limbic |
+ | * [[Limbic encephalitis]]: short-term memory loss, executive dysfuntion, personality changes, panic attacks, and delusions and hallucinations, as well as seizures |
** 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]] |
− | * 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]] |
− | ** CADASIL |
+ | ** [[CADASIL]] |
− | ** Thrombotic thrombocytopenic purpura (TTP) |
+ | ** [[Thrombotic thrombocytopenic purpura]] (TTP) |
** Hyperviscosity |
** Hyperviscosity |
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− | ** Syndromes/paraproteinemias (polycythemia, monocloncal gammopathies) |
+ | ** Syndromes/paraproteinemias ([[polycythemia]], [[Monocloncal gammopathy|monocloncal gammopathies]]) |
− | ** Hypoxic-ischemic encephalopathy |
+ | ** [[Hypoxic-ischemic encephalopathy]] |
* Infectious |
* Infectious |
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− | ** Whipple disease |
+ | ** [[Whipple disease]] |
− | ** Syphilis |
+ | ** [[Syphilis]] |
− | ** Lyme disease |
+ | ** [[Lyme disease]] |
− | ** Subacute sclerosing |
+ | ** [[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 |
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− | ** 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 |
+ | * [[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 |
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− | ** Kuf disease |
+ | ** [[Kuf disease]] |
− | ** Methylmalonic acidemia |
+ | ** [[Methylmalonic acidemia]] |
− | ** Mitochondrial encephalopathies |
+ | ** [[Mitochondrial encephalopathy|Mitochondrial encephalopathies]] |
* Autoimmune |
* Autoimmune |
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− | ** Paraneoplastic limbic encephalitis |
+ | ** [[Paraneoplastic limbic encephalitis]] |
** Anti-VGKC-E |
** Anti-VGKC-E |
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− | ** Hashimoto encephalopathy |
+ | ** [[Hashimoto encephalopathy]] |
− | ** Lupus cerebritis |
+ | ** [[Lupus cerebritis]] |
+ | ** [[Neurosarcoidosis]] |
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− | ** Sarcoid |
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− | ** CNS vasculitis |
+ | ** [[CNS vasculitis]] |
− | ** Celiac disease |
+ | ** [[Celiac disease]] |
* Metastases/neoplastic |
* Metastases/neoplastic |
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** CNS metastases |
** CNS metastases |
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− | ** Primary CNS lymphoma |
+ | ** [[Primary CNS lymphoma]] |
** Intravascular lymphoma |
** Intravascular lymphoma |
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− | ** Lymphomatoid granulomatosis |
+ | ** [[Lymphomatoid granulomatosis]] |
− | ** Gliomatosis cerebri |
+ | ** [[Gliomatosis cerebri]] |
* Iatrogenic/idiopathic |
* Iatrogenic/idiopathic |
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− | ** Central pontine myelinolysis |
+ | ** [[Central pontine myelinolysis]] |
− | ** Insulin-induced hypoglycemia |
+ | ** Insulin-induced [[hypoglycemia]] |
− | ** Normal pressure hydrocephalus (NPH) |
+ | ** [[Normal pressure hydrocephalus]] (NPH) |
* Neurodegenerative |
* Neurodegenerative |
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− | ** |
+ | ** [[Alzheimer dementia]] |
− | ** |
+ | ** [[Creutzfeldt-Jakob disease]] |
* Frontotemporal syndrome |
* Frontotemporal syndrome |
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− | ** Behavioural variant frontotemporal dementia |
+ | ** Behavioural variant [[frontotemporal dementia]] |
** 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]] |
− | ** Dementia with |
+ | ** [[Dementia with Lewy bodies]] |
− | ** Corticobasal degeneration |
+ | ** [[Corticobasal degeneration]] |
− | ** Progressive supranuclear palsy |
+ | ** [[Progressive supranuclear palsy]] |
− | ** Parkinson disease dementia |
+ | ** [[Parkinson disease dementia]] |
** High definition |
** High definition |
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* Systemic |
* Systemic |
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− | ** Vasculitis (giant cell arteritis) |
+ | ** [[Vasculitis]] ([[giant cell arteritis]]) |
− | ** Sleep apnea |
+ | ** [[Sleep apnea]] |
+ | |||
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[[Category:Neurology]] |
[[Category:Neurology]] |
Latest revision as of 14:09, 15 July 2022
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