cerebral arteritis

Cerebral Arteritis: A Rare but Serious Condition

Cerebral arteritis, also known as primary angiitis of the central nervous system (PACNS), is a rare and serious condition that affects the blood vessels in the brain. It is characterized by inflammation and damage to the blood vessels, which can lead to various symptoms and complications.

Symptoms of Cerebral Arteritis

The common symptoms of cerebral arteritis include:

• Headache: A severe and persistent headache is a hallmark symptom of cerebral arteritis [3].
• Confusion: Patients may experience confusion, intellectual impairment, and personality changes [5].
• Seizures: Seizures can occur due to the inflammation and damage to the blood vessels in the brain [5].
• Vision loss: Vision loss or blindness can occur if the condition affects the blood vessels supplying the eyes [14].

Other Symptoms

Other symptoms of cerebral arteritis may include:

• Stroke: A stroke can occur due to the blockage of a blood vessel in the brain [2, 8].
• Encephalopathy: Encephalopathy, which is inflammation of the brain tissue, can also occur [2].
• Cranial nerve palsies or myelopathies: Inflammation and damage to the cranial nerves or spinal cord can lead to various neurological symptoms [2].

Diagnosis

The diagnosis of cerebral arter

Cerebral arteritis, also known as giant cell arteritis or cranial arteritis, can cause a range of signs and symptoms. These may include:

• Headaches: A severe headache is one of the most common symptoms of cerebral arteritis [6][7].
• Stroke-like symptoms: Cerebral arteritis can cause stroke-like episodes, including weakness or numbness in the face or limbs [5][8].
• Difficulty with coordination: Some people may experience difficulty with coordination or balance due to cerebral arteritis [9].
• Confusion: Confusion and changes in mental status are also possible symptoms of cerebral arteritis [6][13].
• Vision problems: Cerebral arteritis can cause vision problems, including blindness if left untreated [2][14].

It's worth noting that the symptoms of cerebral arteritis can vary from person to person, and not everyone will experience all of these symptoms. If you're experiencing any of these symptoms, it's essential to seek medical attention promptly.

References: [1] Not provided [2] 12 [3] Not provided [4] Not provided [5] 5 [6] 6 [7] 7 [8] 8 [9] 9 [10] Not provided [11] Not provided [12] Not provided [13] 13 [14] 14

Cerebral arteritis, also known as giant cell arteritis (GCA), is a condition that affects the blood vessels in the brain and can lead to serious complications if left untreated. Diagnostic tests for cerebral arteritis are crucial in confirming the diagnosis and ruling out other conditions.

Recommended diagnostic tests:

• Blood tests:
  • ESR (erythrocyte sedimentation rate) [1]
  • C-reactive protein [1]
  • Blood count [1]
  • Serum albumin electrophoresis [1]
• Imaging techniques:
  • Angiography [4, 7] allows a good visualization of cerebral vascular structures with low radiation and contrast medium doses as well as short examination times.
  • MRI (magnetic resonance imaging) and CT (computer tomography) scans are usually abnormal but the findings are not specific for PACNS (primary angiitis of the central nervous system) [14]
• Other tests:
  • Nimodipine test during angiography can demonstrate the reversibility of cerebral vasoconstriction syndrome (RCVS) from vasculitis [4]

Diagnostic criteria:

The diagnosis of vasculitis, including CNS vasculitis, is based on a person's medical history, symptoms, and laboratory findings. A complete blood cell count can tell whether you have enough red blood cells. Blood tests that look for certain antibodies — such as the anti-neutrophil cytoplasmic antibody (ANCA) test — can help diagnose vasculitis [8].

Importance of differential diagnosis:

The most important differential diagnosis to CNS vasculitis is RCVS, which is characterized by thunderclap headache, watershed cerebral ischemia, cortical subarachnoidal hemorrhages and angiography suggestive for 'vasculitis' (Table 2). However, RCVS is reversible within 12 weeks and is a non-inflammatory disease which should be treated differently [9].

Confirmation of diagnosis:

The diagnosis had been confirmed by biopsy in just 248 of the 701 published cases of primary CNS vasculitis (35.4%) [10]. In 99 people with vasculitis on biopsy, cerebral angiography was normal.

References:

[1] Tabandeh H. Giant cell arteritis: diagnosis and management. Curr Opin Ophthalmol. 2001;12:393–399. doi: 10.1097/00055735...

[4] by P Berlit · 2010 · Cited by 206 — Laboratory findings suggestive of a systemic vasculitis include an acute inflammatory response with raised erythrocyte sedimentation rate (ESR) and increased...

[7] Primary CNS vasculitis (PCNSV) can be diagnosed using cerebral angiography or histopathology combined with clinical features.

[8] by P Berlit · Cited by 206 — Laboratory tests in suspected vasculitis should search for sys- temic inflammation including specific antibodies, but must also exclude important differential...

[9] The most important differential diagnosis to CNS vasculitis is RCVS, which is characterized by thunderclap headache, watershed cerebral ischemia, cortical subarachnoidal hemorrhages and angiography suggestive for 'vasculitis' (Table 2). However, RCVS is reversible within 12 weeks and is a non-inflammatory disease which should be treated differently.

[10] In a highly informative 2017 systematic study of diagnostic test results in primary CNS vasculitis,27 the authors identified 701 published cases. The diagnosis had been confirmed by biopsy in just 248 of these patients (35.4%).

Treatment Options for Cerebral Arteritis

Cerebral arteritis, also known as giant cell arteritis (GCA), is a chronic inflammatory vasculitis that predominantly affects large- and medium-sized arteries in individuals older than 50. The main goal of treatment is to control the inflammation and prevent complications such as irreversible blindness.

Medications Used to Treat Cerebral Arteritis

• Corticosteroids: High doses of corticosteroid drugs, such as prednisone, are often used to bring the disease under control (establish remission). This is usually done in conjunction with other medications.
• Cyclophosphamide: This medication is sometimes used in combination with corticosteroids to help manage the condition.
• Biologic drugs: In some cases, biologic drugs such as tocilizumab may be prescribed to treat cerebral arteritis.

Treatment Phases

The treatment of cerebral arteritis typically consists of two phases:

1. Induction of remission: This phase involves using high doses of corticosteroids and cyclophosphamide to bring the disease under control.
2. Maintenance therapy: Once the disease is in remission, maintenance therapy may be necessary to prevent relapse.

Other Treatment Options

In addition to medications, other forms of treatment may include:

• Physical, occupational, and speech therapy: These therapies can help manage symptoms such as memory loss and cognitive impairment.
• Lifestyle modifications: Making lifestyle changes such as quitting smoking and exercising regularly can also be beneficial in managing the condition.

References

• [10] Giant cell arteritis (GCA) is a chronic inflammatory vasculitis that predominantly affects large- and medium-sized arteries in individuals older than 50.
• [12] The granulomatous nature of GCA contributes to the loss of vascular smooth muscle cells and elastic fibers, potentially leading to aneurysm formation and other complications.
• [7] Current recommendation is oral prednisone, 1 mg/kg/d, and cyclophosphamide for induction of remission.
• [13] Temporal arteritis treatment should begin immediately to prevent vision loss.

The differential diagnosis of cerebral arteritis, also known as cerebral vasculitis, is broad and includes various conditions that can mimic or coexist with this condition.

Reversible Cerebral Vasoconstriction Syndrome (RCVS): This is a key differential diagnosis for CNS vasculitis, characterized by thunderclap headache, watershed cerebral ischemia, cortical subarachnoidal hemorrhages, and angiography suggestive of "vasculitis" [1]. RCVS is reversible within 12 weeks and is a non-inflammatory disease.

Other conditions to consider: These include degenerative vasopathies, embolic diseases, coagulation disorders, Aspergillosis, Atrial myxoma with embolization to the brain, Behcet Disease, CADASIL (Cerebral Autosomal Dominant Arteriopathy), Moyamoya disease, and inflammatory cerebral amyloid angiopathy [3, 4].

Temporal arteritis: This condition is an important consideration in the differential diagnosis of vascular headaches in adults, particularly those caused by vasculitis [6].

Primary Angiitis of the CNS (PACNS): Differentiation between PACNS and secondary causes is crucial in guiding management, as it affects treatment decisions [15].

Systemic lupus erythematosus: Secondary cerebral vasculitis has been reported in 7-10% of patients with systemic lupus erythematosus, highlighting the importance of considering this condition in the differential diagnosis [11].

Takayasu arteritis: This condition is another important consideration in the differential diagnosis of CNS vasculitis, particularly when evaluating conditions such as cerebral aneurysms and IgG4-related disease [8].

In summary, the differential diagnosis of cerebral arteritis is complex and requires careful consideration of various conditions that can mimic or coexist with this condition.

References:

[1] The most important differential diagnosis to CNS vasculitis is RCVS, which is characterized by thunderclap headache, watershed cerebral ischemia, cortical subarachnoidal hemorrhages and angiography suggestive for ‘vasculitis’ (Table 2).

[3] Differential Diagnoses · Aspergillosis · Atrial myxoma with embolization to the brain · Behcet Disease · CADASIL (Cerebral Autosomal Dominant ...

[4] 3 days ago — Differential diagnosis · reversible cerebral vasoconstriction syndromes (RCVS) · moyamoya disease · inflammatory cerebral amyloid angiopathy.

[6] Headaches caused by vasculitis, especially temporal arteritis, are important in the differential diagnosis of vascular headaches in adults.

[8] Oct 29, 2024 — Conditions to consider in the differential diagnosis of Takayasu arteritis include the following: Cerebral aneurysms. IgG4-related disease.

[11] Secondary cerebral vasculitis in postmortem studies has been reported in 7-10% of patients with systemic lupus erythematosus.

[15] Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations.