Neuro‐ophthalmological disorders in cerebral palsy: ophthalmological, oculomotor, and visual aspects

Abstract

AIM Cerebral visual impairment (CVI) is a disorder caused by damage to the retrogeniculate visual pathways. Cerebral palsy (CP) and CVI share a common origin: 60 to 70% of children with CP also have CVI. We set out to describe visual dysfunction in children with CP. A further aim was to establish whether different types of CP are associated with different patterns of visual involvement.METHODS A total of 129 patients (54 females, 75 males; mean age 4y 6mo, SD 3y 5mo; range 3mo-15y) with CP (51 with diplegia, 61 with tetraplegia, and 17 with hemiplegia; 62 [48%] of participants were able to walk) and CVI enrolled at the Centre of Child Neuro-ophthalmology (at the Department of Child Neurology and Psychiatry, IRCCS 'C. Mondino Institute of Neurology', University of Pavia) underwent an assessment protocol including neurological examination, developmental and ⁄ or cognitive assessment, neuro-ophthalmological evaluation including ophthalmological assessment, evaluation of visual acuity, contrast sensitivity, optokinetic nystagmus, visual field and stereopsis, and neuroradiological investigations.

Neuro-ophthalmological emergency disorders: A general view

“…Symptoms associated with neuro-ophthalmic disorders usually include afferent visual system disorders such as optic neuritis (especially Multiple Sclerosis (MS)), vision-related migraines, optic neuropathy, papilledema, pseudotumor cerebri, brain tumors or strokes, and the efferent visual system disorders such as anisocoria and other pupil abnormalities, diplopia and other visual disturbances (phospenes, etc. ), un-explained vision loss, sudden temporary or permanent visual loss, ophthalmoplegia, ptosis, eyelid and facial spasms, eye movement disorders in paralitic or restrictive types (thyroid eye disease, Myasthenia gravis, nystagmus, blepharospasm, and acute visual perception disorders or high cortical visual dysfunctions [1][2][3][4][5][6][7][8][9]. However, the neuro-ophthalmological emergencies constitute vision or life-threatening conditions if diagnosis and treatments are not made promptly.…”

“…However, the neuro-ophthalmological emergencies constitute vision or life-threatening conditions if diagnosis and treatments are not made promptly. The common neuro-ophthalmological emergency disorders are arteritic anterior ischemic optic neuropathy (AAION), pituitary apoplexy, cavernous sinus thrombosis, rino-orbital-cerebral mucormycosis (ROCM), isolated third nerve palsy, multiple unilateral or bilateral oculomotor palsies, acute methanol toxication, Tolosa-Hunt syndrome, Miller Fisher Syndrome and Horner syndrome [1][2][3][4][5][13][14][15]. The main signs of the life or sight-threatening neuro-ophthalmological emergency conditions include diplopia, ptosis (acute painful or chronic painless), transient visual loss, anisocoria, acute painful or painless homonymous hemianopsia, acute bitemporal hemianopsia, severe pain in head/neck, painful ophthalmoplegia and ocular motility disorder [1][2][3][4][5][6][7][8][9].…”

“…Because the compressive lesions of CN3 usually affect both the central somatomotor ibers and the peripheral superomedial pupillary ibers and they cause the pupil-involving CN3 palsy. However, ischemic lesions of the nerve involve only the central somatomotor ibers, so, ischemic CN3 palsy has no a pupillary involvement [1][2][3][4][5][6][7][8][9]. The main mechanisms of the aneurysmal CN3 palsy, including direct compression by an enlarged aneurysmal sac, subarachnoid blood irritation, or aneurysm pulsation.…”

“…Besides an aneurysm and vascular pathology, giant cell arteritis (GCA), pituitary apoplexy, demyelinating disease (MS), midbrain infarction, CN3 schwannoma/meningioma or cerebral metastasis, trauma and ophthalmoplegic migraine may also cause the CN3 palsy with pupil involvement. Although Myasthenia gravis (MG) may mimic a CN3 palsy, the pupil is never be involved in MG [1][2][3][4][5][6][7][8][9][10][11][12]. Myasthenia gravis is a neuromuscular disease leading to increasing muscle weakness and fatigue at the end of the day.…”

“…Thus, AAION is another neuro-ophthalmological and medical emergency disease. Because AAION forms 10% of all patients with anterior ischemic optic neuropathy, and it has about a 75% risk of the blindness in both eyes within days or weeks when AAION is not diagnosed recognized and treated promptly [1][2][3][4][5][6][7][8][9][10][11].…”