Definition

The term “inflammatory cerebral amyloid angiopathy” can be used as an umbrella term encompassing two subtypes: cerebral amyloid angiopathy-related inflammation and amyloid β-related angiitis 2,6. However, many authors interchange the terms “cerebral amyloid angiopathy-related inflammation” and “inflammatory cerebral amyloid angiopathy,” either encompassing of amyloid β-related angiitis 8 or in distinction to it 3. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10 or amyloid β-related angiitis alone 7 without mention of the other.

When the distinction is made, the difference lies in whether the inflammation is perivascular only (cerebral amyloid angiopathy-related inflammation or inflammatory cerebral amyloid angiopathy) or also involves and destroys the vessel wall (amyloid β-related angiitis).

This pathological distinction is not reliably predicted on imaging 2. It may also be possible that, due to sampling error on biopsy, the pathological diagnosis does not reflect the global picture depicted on imaging 6. For these reasons, this article does not attempt to distinguish between subtypes and treats the terms interchangably.

Other synonyms used for this entity include cerebral amyloid inflammatory vasculopathy, amyloid angiopathy and granulomatous angiitis of the central nervous systemcerebral amyloid angiitis, primary angiitis of the central nervous system associated with cerebral amyloid angiopathy, and cerebral amyloid angiopathy associated with giant cell arteritis 9.

Additionally, although there is considerable overlap, inflammatory cerebral amyloid angiopathy should be distinguished from amyloid-related imaging abnormalities (ARIA) that are seen in the setting of treatment with novel amyloid-lowering therapies such as monoclonal antibodies 1

Etiopathogenesis

  • the optic nerve is sheathed in all three meningeal layers and is continuous with the subarachnoid space of the brain
  • intracranial CSF is by this way contiguous with the cerebrospinal fluid (CSF) surrounding the optic nerve, and elevated ICP can be transmitted directly to the optic nerve
  • the pathogenesis of papilledema involves compression of nerve fibers by increased CSF pressure in the subarachnoid space
  • normal pressure gradient across the intraocular and orbital optic nerve is disrupted, causing retrograde axoplasmic flow across the optic disc, which results in disc edema and optic neuropathy (Trobe, 2011)
  • venous stasis, telangiectasia, and hypoxia in the nerve fiber layer are secondary

Clinical Presentation

The clinical presentation is usually acute or subacute 1,2, but may be chronic 4. The diagnostic criteria for possible or probable inflammatory cerebral amyloid angiopathy require at least one of the following clinical features that are not directly attributable to an acute intracerebral hemorrhage 4:

  • headache
  • decrease in consciousness
  • behavioral change
  • focal neurological signs and seizures

Some patients also present with hallucinations 2.

Ophthalmologic Findings

  • typically bilateral, usually symmetrical ophthalmoscopic findings (asymmetry may occur in the early stages)
  • edema with indistinct disc margins, obliteration of the physiologic excavation, venous stasis, and occasional peripapillary flame-shaped hemorrhages
  • dilated and tortuous veins and narrowed arteries
  • if the cause is not resolved and the papilledema progresses, hemorrhages and soft exudates become more prominent, and concentric or radial folds (Paton lines) may be observed on the retina
  • hemorrhages and exudates are resorbed during the transition to the chronic phase
  • in long-term hypertension, papilledema leads to optic nerve atrophy, edema, and hyperemia recede, the disc changes color to greyish white, and the vessels constrict

Foster-Kennedy Syndrome (FKS)

  • characterized by anosmia and visual loss (central scotoma), which may be unilateral or bilateral depending on the stage of the disease
  • it is defined by compressive optic nerve damage (atrophy) in one eye and contralateral papilledema, resulting from increased intracranial pressure secondary to an intracranial space-occupying lesion
  • there are typically three types of FKS
    • type 1 – the most common form with optic atrophy in the ipsilateral eye and papilledema in the contralateral eye
    • type 2 – bilateral papilledema and unilateral optic atrophy
    • type 3 – bilateral papilledema progressing to bilateral optic atrophy

Diagnostic evaluation

Therapy

  • therapy is based on the identified etiology
    • papilledema due to intracranial hypertension should address the cause of raised ICP (antiedema medications, surgery)
    • venous sinus thrombosis requires anticoagulation or local intrasinus therapy
  • if no structural or other causes of intracranial hypertension are detected, the term idiopathic intracranial hypertension is used
    • acetazolamide is administered + weight loss is recommended; venous sinus stenting is considered in patients with IIH who have significant transverse venous sinus stenosis
  • symptomatic surgical procedures
    • optic nerve sheath fenestration (ONSF) is considered when vision is thought to be severely threatened
    • ventriculoperitoneal and lumboperitoneal shunts 

Prognosis

  • the prognosis for papilledema varies depending on the underlying cause and duration of the edema
  • timely intervention can lead to resolution; delayed treatment may result in permanent visual impairment
  • závratě a synkopy (> 50%)
  • ischemické CMP
  • ICH (v ruptury důsledku aneuryzmat)
  • zřídka poruchy vizu (poruchy ZP, amaurosis, retinální hemoragie)
  • sekundární hypertenze (z postižení renálních arterií)
  • diagnostické je oslabení pulsu (“bezpulsová nemoc“), rozdíl na HKK > 20mm Hg
    • 98%  pacientů má chybějící puls na alespoň jedné končetině
  • Raynaudův fenomén
  • končetinové klaudikace
  • aortální regurgitace (20-25%)
  • hypertensive arteriolopathy is the most common cause of intracerebral hemorrhage
  • the relative risk of ICH in a patient with arterial hypertension compared to an individual without hypertension is approximately 4
  • hypertension leads to bleeding by two mechanisms:
    • rupture of an artery affected by chronic hypertension
    • an acute or subacute severe hypertension leading to rupture of a previously unaffected artery (malignant hypertension)
  • typical localization: basal ganglia, thalamus, cerebellum, pons   Typical localisations of hypertonic bleeding  Lenticulo-striate arteries
    • a secondary propagation of hematoma into the ventricles (hemocephalus) or SA space is possible
  • hypertension leads to hypertrophy and degeneration of the media of small arteries (lipohyalinosis, fibrinoid necrosis)
  • the findings suggestive of a hypertensive etiology:
    • history of hypertension
    • typical ICH localization
    • absence of any other apparent cause of bleeding
    • left ventricular hypertrophy
    • leukoaraiosis on CT scan or MRI   Hypertensive small vessel disease (microangiopathy)
    • hypertensive retinopathy
    • high blood pressure on admission is not a conclusive indicator of hypertensive disease; it can be a consequence of a stress reaction and intracranial hypertension

Imunosupresiva – cytostatika 

  • cca 50-70% pacientů vyžaduje doplňující léčbu imunosupresivy
  • u méně agresivních forem:
    • azathioprin (IMURAN)  1-2 mg/kg/den
    • mykofenolat (CELLCEPT)
  • u agresivních forem:
    • metotrexát   0.15-3 mg/kg/den
    • cyklofosfamid (ENDOXAN)  2mg/kg/den

Nová imunosupresiva  [Mekinian, 2015]

    • monoklonální protilátka proti receptoru pro IL 6 tocilizumab (ROACTEMRA)
    • TNF alfa blokátor – u rezistentních stavů

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