π§΅1/ Let's dive into the rare and often misdiagnosed condition: Primary Central Nervous System (CNS) Vasculitis with this article from NEJM. nejm.org
"Primary CNS Vasculitis = The MAD HATTER of the brain! π©π§ Why did we pick this visual mnemonic? This rare condition creates chaos:
Inflammation disrupts the brainβs 'tea party' (vascular network).
Confusion & cognitive dysfunction mirror the Hatterβs erratic behavior.
Unpredictable symptoms make it just as tricky to diagnose as his riddles. If you want to learn more lets go deeper. #MedEd #Neurology"
"Primary CNS Vasculitis = The MAD HATTER of the brain! π©π§ Why did we pick this visual mnemonic? This rare condition creates chaos:
Inflammation disrupts the brainβs 'tea party' (vascular network).
Confusion & cognitive dysfunction mirror the Hatterβs erratic behavior.
Unpredictable symptoms make it just as tricky to diagnose as his riddles. If you want to learn more lets go deeper. #MedEd #Neurology"
2/ A RARE FORM of vasculitis limited to the brain and spinal cord, causing a variety of neurologic syndromes. Estimated annual incidence: 2.4 cases per 1 million person-years. #Vasculitis #Neurology x.com
5/ Other common features include:
HEADACHE: Severe, persistent, generalized or localized (rarely thunderclap headaches)
Progressive cognitive decline
Acute or subacute encephalopathy #Headache #CognitiveImpairment x.com
HEADACHE: Severe, persistent, generalized or localized (rarely thunderclap headaches)
Progressive cognitive decline
Acute or subacute encephalopathy #Headache #CognitiveImpairment x.com
6/ Less common manifestations:
SEIZURE
Intracerebral hemorrhage
Subarachnoid hemorrhage
Spinal cord involvement (myelopathy)
Systemic symptoms like fever and weight loss (infrequent) #Epilepsy #Myelopathy x.com
SEIZURE
Intracerebral hemorrhage
Subarachnoid hemorrhage
Spinal cord involvement (myelopathy)
Systemic symptoms like fever and weight loss (infrequent) #Epilepsy #Myelopathy x.com
8/ SUBSETS of Primary CNS Vasculitis:
Small-vessel vasculitis
Medium-to-large-vessel vasculitis
Each has distinct clinical features, diagnostic methods, and outcomes. #Vasculitis #Neurology x.com
Small-vessel vasculitis
Medium-to-large-vessel vasculitis
Each has distinct clinical features, diagnostic methods, and outcomes. #Vasculitis #Neurology x.com
9/ Small-Vessel Vasculitis:
Often requires brain biopsy for diagnosis
Angiography typically normal
Clinical features: cognitive decline, headaches #Diagnostics #Neuropathology x.com
Often requires brain biopsy for diagnosis
Angiography typically normal
Clinical features: cognitive decline, headaches #Diagnostics #Neuropathology x.com
10/ Medium-to-Large-Vessel Vasculitis:
Detected with cerebral angiography
Shows smooth-wall segmental stenosis of multiple cerebral arteries
Patients present with acute focal neurologic deficits and multiple cerebral infarctions #Radiology #Stroke x.com
Detected with cerebral angiography
Shows smooth-wall segmental stenosis of multiple cerebral arteries
Patients present with acute focal neurologic deficits and multiple cerebral infarctions #Radiology #Stroke x.com
12/ High-Resolution MRI of Vessel Wall:
Reveals segmental, concentric, homogeneous enhancement
Helps differentiate from atherosclerosis and RCVS #Radiology #VesselWallImaging x.com
Reveals segmental, concentric, homogeneous enhancement
Helps differentiate from atherosclerosis and RCVS #Radiology #VesselWallImaging x.com
13/ CSF Analysis:
Mildly increased leukocyte count (>5 cells/mmΒ³)
Increased protein (>45 mg/dL)
Occurs in ~75% of patients, especially in small-vessel vasculitis
Nonspecific but helps rule out other conditions #CSF #LumbarPuncture x.com
Mildly increased leukocyte count (>5 cells/mmΒ³)
Increased protein (>45 mg/dL)
Occurs in ~75% of patients, especially in small-vessel vasculitis
Nonspecific but helps rule out other conditions #CSF #LumbarPuncture x.com
14/ Diagnosis: Relies on:
Clinical evaluation
Imaging studies (MRI, MRA, CTA, digital subtraction angiography)
CSF analysis
Often brain biopsy
Exclusion of alternative diagnoses #Diagnosis #Neurology x.com
Clinical evaluation
Imaging studies (MRI, MRA, CTA, digital subtraction angiography)
CSF analysis
Often brain biopsy
Exclusion of alternative diagnoses #Diagnosis #Neurology x.com
15/ Differential Diagnoses Include:
Reversible cerebral vasoconstriction syndrome (RCVS)
Intracranial atherosclerosis
INTRAVASCULAR LYMPHOMA (The Cheshire Cat)
Moyamoya disease
Secondary cerebral vasculitis (connective-tissue diseases)
Infections #DifferentialDiagnosis
Lymphoma = The Cheshire Cat of cancers: stealthy, unpredictable, and systemic. Disappears mysteriously, much like lymphoma can remain undetected for a long time before symptoms arise It lurks quietly, appearing when least expectedβbut with vigilance and treatment, we can outsmart it. π±β¨
Reversible cerebral vasoconstriction syndrome (RCVS)
Intracranial atherosclerosis
INTRAVASCULAR LYMPHOMA (The Cheshire Cat)
Moyamoya disease
Secondary cerebral vasculitis (connective-tissue diseases)
Infections #DifferentialDiagnosis
Lymphoma = The Cheshire Cat of cancers: stealthy, unpredictable, and systemic. Disappears mysteriously, much like lymphoma can remain undetected for a long time before symptoms arise It lurks quietly, appearing when least expectedβbut with vigilance and treatment, we can outsmart it. π±β¨
16/ Brain Biopsy:
Gold standard for diagnosis
Provides definitive evidence of vasculitis
Invasive with potential complications
Reserved for uncertain cases #Neuropathology #Biopsy x.com
Gold standard for diagnosis
Provides definitive evidence of vasculitis
Invasive with potential complications
Reserved for uncertain cases #Neuropathology #Biopsy x.com
17/ Histopathological Patterns:
Granulomatous (GRANNY) vasculitis (most common)
Lymphocytic vasculitis
Necrotizing vasculitis
These patterns typically remain stable over time in a patient. #Pathology #Neurology x.com
Granulomatous (GRANNY) vasculitis (most common)
Lymphocytic vasculitis
Necrotizing vasculitis
These patterns typically remain stable over time in a patient. #Pathology #Neurology x.com
18/ Treatment Overview: (LETS CHANGE THE SUBJECY)
Immunosuppressive therapy is a mainstay
Glucocorticoids are often combined with cyclophosphamide
Early treatment can prevent serious outcomes #Treatment #Immunology x.com
Immunosuppressive therapy is a mainstay
Glucocorticoids are often combined with cyclophosphamide
Early treatment can prevent serious outcomes #Treatment #Immunology x.com
19/ Glucocorticoids:
Oral prednisone at 1 mg/kg/day
May start with IV methylprednisolone (1g daily for 3β5 days)
Tapered based on response and side effects
"Prednisone = Fred Flintstone! 𦴠Why?
Phonetic match: Fred & Pred.
Increased appetite π? Just like Fred at dinner!
Mood swings π? That classic Fred temper.
Strong anti-inflammatory punch πͺ? Yabba Dabba Dooo!π§ #MedEd #Pharmacology"
#Steroids #Pharmacology
Oral prednisone at 1 mg/kg/day
May start with IV methylprednisolone (1g daily for 3β5 days)
Tapered based on response and side effects
"Prednisone = Fred Flintstone! 𦴠Why?
Phonetic match: Fred & Pred.
Increased appetite π? Just like Fred at dinner!
Mood swings π? That classic Fred temper.
Strong anti-inflammatory punch πͺ? Yabba Dabba Dooo!π§ #MedEd #Pharmacology"
#Steroids #Pharmacology
20/ Cyclophosphamide: (CYCLOPS red PHOSPHORUS)
Administered as monthly IV pulses (0.5β1.0 g/mΒ²) for 6 months
Used in severe or rapidly progressive cases
Aims to induce remission and reduce glucocorticoid dependence #Chemotherapy #Immunosuppression x.com
Administered as monthly IV pulses (0.5β1.0 g/mΒ²) for 6 months
Used in severe or rapidly progressive cases
Aims to induce remission and reduce glucocorticoid dependence #Chemotherapy #Immunosuppression x.com
21/ Alternative Therapies:
Mycophenolate mofetil (2β3 g/day): As effective as cyclophosphamide in some studies; less toxic
Mycophenolate = Michael Flatley of immunosuppressants:
π Immune system out of rhythm? Myco brings order like Flatley leading Lord of the Dance.
π― Targeted action calms the chaos, preventing autoimmune flares or transplant rejection. #MedEd #Pharmacology"
Rituximab and Tocilizumab: Options for refractory cases or contraindications #Biologics #Rheumatology
Mycophenolate mofetil (2β3 g/day): As effective as cyclophosphamide in some studies; less toxic
Mycophenolate = Michael Flatley of immunosuppressants:
π Immune system out of rhythm? Myco brings order like Flatley leading Lord of the Dance.
π― Targeted action calms the chaos, preventing autoimmune flares or transplant rejection. #MedEd #Pharmacology"
Rituximab and Tocilizumab: Options for refractory cases or contraindications #Biologics #Rheumatology
22/ Maintenance Therapy:
After induction, maintenance with mycophenolate mofetil or azathioprine
Associated with lower relapse rates and better outcomes
Duration: At least 2 years suggested #LongTermCare #Pharmacology x.com
After induction, maintenance with mycophenolate mofetil or azathioprine
Associated with lower relapse rates and better outcomes
Duration: At least 2 years suggested #LongTermCare #Pharmacology x.com
23/ Monitoring for Side Effects:
Risk of infection due to immunosuppression
Prophylaxis for Pneumocystis pneumonia with TMP-SMX recommended
Monitor bone health; consider osteoporosis prophylaxis #PatientSafety #InfectionControl x.com
Risk of infection due to immunosuppression
Prophylaxis for Pneumocystis pneumonia with TMP-SMX recommended
Monitor bone health; consider osteoporosis prophylaxis #PatientSafety #InfectionControl x.com
24/ Prognosis:
Induction therapy leads to remission in 68β95%
Flares occur in 12β59%
Good functional outcomes (modified Rankin scale β€2) in 46β73% #Prognosis #Outcomes x.com
Induction therapy leads to remission in 68β95%
Flares occur in 12β59%
Good functional outcomes (modified Rankin scale β€2) in 46β73% #Prognosis #Outcomes x.com
Reversible Cerebral Vasoconstriction Syndrome (RCVS) = Obama of neurology: πΊπΈπ§
Sudden thunderclaps = major political crises (think healthcare reform).
Vasospasm reversibility = Obamaβs calm ability to course-correct.
Intense start, but resolves with time & patience = just like his presidency.
RCVS: intense but reversible, just like Obamaβs journey in the White House! #MedEd #Neurology
Sudden thunderclaps = major political crises (think healthcare reform).
Vasospasm reversibility = Obamaβs calm ability to course-correct.
Intense start, but resolves with time & patience = just like his presidency.
RCVS: intense but reversible, just like Obamaβs journey in the White House! #MedEd #Neurology
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