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Radiological Interpretation in Neuro-Ophthalmology
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Chirag Kamal Ahuja, Paramjeet Singh
The neural is consequent to variable degree of partial/complete involvement of one/combination of CN III, IV, VI, V1 or V2 segments of the V CNs. The patients may present with ophthalmoparesis (ptosis, diplopia), restriction of ocular movements, reduced vision and weakness or pain in trigeminal distribution. Neural involvement may also occur due to inherent nerve paresis (secondary to diabetes-related neuropathy or microvascular affliction of neural supply) or selective neural compression like PCom aneurysm causing selective third nerve compression in the supracavernous region (Figure 22.20). Vascular manifestations may be secondary to venous congestion due to cavernous sinus invasion leading to proptosis (pulsatile in CCF), chemosis, pain on ocular movements, etc. Orbital CT with cavernous sinus cuts is sufficient for the evaluation most of the times. Sometimes, MR has to be performed when CT is unable to detect very small lesions and characterize them.
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
Idiopathic PD, distinguished from PSP by: More commonly asymmetric onset.Less immobile facies without lid retraction.Blink rate not so reduced until advanced disease.Absence of supranuclear ophthalmoparesis, particularly downgaze.Absence of early pronounced gait imbalance.Absence of signs of pseudobulbar palsy (such as harsh, strained voice quality of spastic dysarthria) until late, if at all.More appendicular than axial rigidity and bradykinesia.Tremor is usually present; PSP tremor may occur in 5–10% of cases but is either absent, not pronounced, or of low amplitude.Responsiveness to levodopa.
Kearns-Sayre Syndrome
Published in Shamim I. Ahmad, Handbook of Mitochondrial Dysfunction, 2019
The first symptom and sign in KSS is usually ptosis, recognized when children use their brow muscle to elevate the eyelids (Park et al., 2004). Later in the disease course, PEO and other phenotypic features may develop (Park et al., 2004). PEO usually starts after age 5y and typically after onset of ptosis (Park et al., 2004). PEO usually affects horizontal and upward gaze but frequently spares downward gaze (Simaan et al., 1999). Diplopia is unusually due to the slow progression of PEO (Park et al., 2004). Only occasionally, easy fatigability and myopathy of the limb muscles may be seen (Park et al., 2004; van Beynum et al., 2012). Some patients with failure to thrive or dilated cardiomyopathy as initial manifestations have been reported (van Beynum et al., 2012). In a study of 19 Chinese patients with KSS, ophthalmoparesis was the presenting feature at onset in 16 patients. In three patients, short stature was the initial manifestation (Yu et al., 2016). Mean age at onset in this cohort was 9.6y (Yu et al., 2016).
Ophthalmological Manifestations of Hereditary Myopathies
Published in Journal of Binocular Vision and Ocular Motility, 2022
Marta Saint-Gerons, Miguel Angel Rubio, Gemma Aznar, Ana Matheu
Congenital myopathies are a clinically and genetically heterogeneous group of muscle diseases that begin generally in childhood. They present with motor weakness, hypotonia, and motor development delay with a static or slow progression. The ophthalmological features include ptosis and ophthalmoparesis. Congenital myopathies can be classified based on their histology. The five types are centronuclear myopathy (subtypes: myotubular myopathy and autosomal centronuclear myopathy), nemaline myopathy, core myopathy, myosin storage myopathy, and congenital fiber-type disproportion.1In the last 15 years, more than 20 genes causing these diseases have been identified.2 These genes are implicated in an abnormal excitation-contraction coupling, malformation of contractile filaments, or regulation of calcium homeostasis.3,4 However, there is an important overlap, as different mutations in the same gene can cause distinct muscle pathologies, and mutations in different genes can cause very similar phenotypic defects5,6 (Table 1).
Neuro-Ophthalmic Literature Review
Published in Neuro-Ophthalmology, 2021
David A. Bellows, Noel C.Y. Chan, John J. Chen, Hui-Chen Cheng, Peter W. MacIntosh, Jenny A. Nij Bijvank, Michael S. Vaphiades, Konrad P. Weber, Sui H. Wong
Ocular MG: Ophthalmoparesis or ptosis in ocular MG that is not responding to anticholinesterase agents should be treated with immunosuppressant (IS) agents if symptoms are functionally limiting or troublesome to the patient.Corticosteroids should be used as the initial IS agent in ocular MG and steroid sparing agents may be considered when corticosteroids are ineffective, contraindicated or not tolerated.Low dose corticosteroids may be effective for ocular MG and may avoid side effects associated with higher doses.ACHR positive patients with ocular MG who do not respond adequately to acetylcholinesterases and who prefer not to take IS therapy or have contraindications or are refractory may be offered thymectomy
Photophobia as the Presenting Symptom of Internal Carotid Artery Dissection
Published in Neuro-Ophthalmology, 2020
Francesco Pellegrini, Daniele Cirone, Altin Stafa
Neck and facial pain, headache, pulsatile tinnitus, amaurosis fugax and retinal ischaemia may all occur in isolation or in various combinations when the ICA is involved.4 HS, characterised by ptosis and miosis, is caused by compression of the ascending sympathetic supply within the carotid sheath and occurs in fewer than half of patients affected by ICA dissection,5 although it is the most common neuro-ophthalmological manifestation.6 ICA dissection should always be considered in the setting of painful HS, i.e., when associated with ipsilateral headache or pain, carotidynia, as well as signs and symptoms consistent with ipsilateral ocular or cerebral ischaemia.7 Another common symptom of neuro-ophthalmological interest is amaurosis fugax, while uncommon and rare manifestations include: anterior and posterior ischaemic optic neuropathy; central retinal artery occlusion; ophthalmic artery occlusion; transient ophthalmoparesis; third, fourth, or sixth nerve palsy; ocular ischaemic syndrome; homonymous field defects; and monocular scintillation.6