Introduction

Epidemiology

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.

Etiopathogenesis

Ophthalmologic Findings

Ophthalmologic Findings

Ophthalmologic Findings

Clinical Presentation

Diagnostic evaluation

Differential diagnosis

Management

Therapy

Prognosis

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
  • 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
  • compensate blood pressure (BP) in all ICH patients, start as soon as possible after bleeding
    • combine pharmacological and non-pharmacological approaches
  • target BP: < 130/80 mm Hg
  • reduce salt uptake
  • avoid smoking, alcohol and drug abuse
  • treat sleep apnea if present
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