CADASIL or CADASIL syndrome, involving cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, is the most common form of hereditary stroke disorder, and is thought to be caused by mutations of the Notch 3 gene on chromosome 19. The disease belongs to a family of disorders called the leukodystrophies. The most common clinical manifestations are migraine headaches and transient ischemic attacks or strokes, which usually occur between 40 and 50 years of age, although MRI is able to detect signs of the disease years prior to clinical manifestation of disease.The condition was identified and named by French researchers Marie-Germaine Bousser and Elisabeth Tournier-Lasserve in the 1990s. Together with two other researchers, Hugues Chabriat and Anne Joutel, they received the 2019 Brain Prize for their research into the condition. Signs and symptoms
CADASIL may start with attacks of migraine with aura or subcortical transient ischemic attacks or strokes, or mood disorders between 35 and 55 years of age. The disease progresses to subcortical dementia associated with pseudobulbar palsy and urinary incontinence.Ischemic strokes are the most frequent presentation of CADASIL, with approximately 85% of symptomatic individuals developing transient ischemic attacks or stroke(s). The mean age of onset of ischemic episodes is approximately 46 years (range 30–70). A classic lacunar syndrome occurs in at least two-thirds of affected patients while hemispheric strokes are much less common. It is worthy of note that ischemic strokes typically occur in the absence of traditional cardiovascular risk factors.
Endophthalmitis is inflammation of the interior cavity of the eye, usually caused by infection. It is a possible complication of all intraocular surgeries, particularly cataract surgery, and can result in loss of vision or loss of the eye itself. Infection can be caused by bacteria or fungi, and is classified as exogenous (infection introduced by direct inoculation as in surgery or penetrating trauma), or endogenous (organisms carried by blood vessels to the eye from another site of infection). Other non-infectious causes include toxins, allergic reactions, and retained intraocular foreign bodies. Intravitreal injections are a rare cause, with an incidence rate usually less than .05%. Signs and symptoms
There is usually a history of recent eye surgery or penetrating trauma to the eye. Symptoms include severe pain, vision loss, and intense redness of the conjunctiva. Hypopyon can be present and should be looked for on examination by a slit lamp. It can first present with the black dot sign (Martin-Farina sign), where patients may report a small area of loss of vision that resembles a black dot or fly. An eye exam should be considered in systemic candidiasis, as up to 3% of cases of candidal blood infections lead to endophthalmitis. Complications
Panophthalmitis — Progression to involve all the coats of the eye. Corneal ulcer
Orbital cellulitis
Impairment of vision
Complete loss of vision
Loss of eye architecture
Enucleation

Cause
Bacteria: N. meningitidis, Staphylococcus aureus, S. epidermidis, S. pneumoniae, other streptococcal spp., Cutibacterium acnes, Pseudomonas aeruginosa, other gram negative organisms. Viruses: Herpes simplex virus. Fungi: Candida spp.