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2.1
Familial Congenital Palsy of Trochlear Nerve

Presentation

Four contiguous, 1-mm-thick MRI oblique axial image planes, obtained with heavy T 2 -weighting, are presented in rostral-to-caudal sequence. F igure 4. [iovs.arvojournals.org]

The 7-year-old presented with congenital ptosis and restricted eye movements. At the age of 2 years she had bilateral ptosis repair. [cags.org.ae]

At postoperative 12 months, all patients had motor success in the present group. [journals.plos.org]

There was no significant difference of the SO volume ratio between controls and the present group. [ajnr.org]

[…] third cranial nerve palsies may fall into the realm of CCDDs sharing much wider spectrum of presentation with involvement of the MLF as well as nuclei of trigeminal nerve. [ijo.in]

Treatment

He is currently involved in multiple clinical trials of novel genetic interventions for the treatment of spinal muscular atrophy and Duchenne muscular dystrophy. [books.google.com]

A Cochrane Systematic Review compared several surgical treatments (myectomy, recession, anterior transposition, disinsertion) in people with fourth nerve palsy. [7] While there was not enough high-quality evidence to recommend the best surgical treatment [en.wikipedia.org]

We've obviously reborrowed the full form "paralysis" into English as well; today ...Read more 12 What is the treatment for median nerve entrapment? Depends on severity! [healthtap.com]

[…] of Bell's palsy Statistical degrees of benefit from drug treatment Prednisone > Placebo More benefit when treatment started within 3 days of onset No benefit from treatment starting more than 10 days after onset Corticosteroids Use within one week of [neuromuscular.wustl.edu]

Patients who underwent IO myectomy as the first surgical treatment were included. [journals.plos.org]

Prognosis

The prognosis of a fourth nerve palsy depends on the underlying etiology. [emedicine.medscape.com]

Prognosis When a child presents with a postinfectious, isolated trochlear palsy that cannot be explained as congenital, traumatic, restrictive, myasthenic, or neoplastic, the prognosis is good and observation alone is sufficient. [guwsmedical.info]

[…] titers Electrodiagnositic testing: In severe paresis to determine extent of injury Variant: Bilateral simultaneous facial palsy 12 Frequency: 0.4% of Bells palsy Onset in 2nd nerve: 1 to 6 days after 1st nerve paresis Severity: Often severe bilaterally Prognosis [neuromuscular.wustl.edu]

Traumatic facial palsy in neonates is associated with good prognosis as seen in the index case. In contrast nontraumatic facial palsies carry a poor functional outcome [ 13, 14 ]. [ispub.com]

PATOLOGIA NEOPLASTICA min. max. fisso 9322 NEOPLASIE A PROGNOSI FAVOREVOLE CON MODESTA COMPROMISSIONE FUNZIONALE / / 11 9323 NEOPLASIE A PROGNOSI FAVOREVOLE CON GRAVE COMPROMISSIONE FUNZIONALE / / 70 9325 NEOPLASIE A PROGNOSI INFAUSTA O PROBABILMENTE [medisoc.it]

Etiology

SO hypoplasia of ≤75% compared with the contralateral normal side suggests trochlear nerve absence as the etiology of congenital SOP. Footnotes H.K. Yang and D.S. Lee contributed equally to this work. [ajnr.org]

Incidence and Etiology of Presumed Fourth Cranial Nerve Palsy: A Population-based Study. Am J Ophthalmol. 2018 Jan. 185:110-114. [Medline]. Richards BW, Jones FR Jr, Younge BR. [emedicine.medscape.com]

The most common etiology was congenital (94.9%). Knapp’s Type III (66.7%) and Type I (12.8%) classifications were the most common subtypes. [ijo.cn]

Etiology of Fourth Nerve Palsy. [guwsmedical.info]

Practically speaking, the specific muscle(s) involved and the etiology of a vertical strabismus not due to a fourth nerve palsy is often not resolved by the 3-step plus fourth step test, because acquired vertical strabismus is often the result of the [aao.org]

Epidemiology

[…] lymphoblastic leukemia Spinocerebellar ataxia type 1 Spondylocarpotarsal synostosis Synonym(s): - Inherited congenital spastic quadriplegia Classification (Orphanet): - Rare genetic disease - Rare neurologic disease Classification (ICD10): (no data available) Epidemiological [csbg.cnb.csic.es]

[…] tightly together: Difficulty keeping food in mouth Facial muscle atrophy (Late) Electrophysiology EMG Denervation Synkinesis: Late Blink reflex Abnormal ipsilateral R1 (early, disynaptic) R2 (late multisynaptic) responses Synkinesis (Late) Bell's Palsy 8 Epidemiology [neuromuscular.wustl.edu]

Epidemiology Frequency Most cases of isolated fourth nerve palsy are believed to be congenital. [4] However, estimating the true frequency of congenital fourth nerve palsy is difficult. [emedicine.medscape.com]

Pathophysiology

Her research aims to identify genetic aetiologies for a range of inherited diseases (monogenic types of diabetes and vertebral dysegmentation disorders in addition to CCDDs) in order to understand the underlying pathophysiology and provide genetic testing [yumpu.com]

Pinealoma Para Myokymia of the superior oblique muscle (typically with brief episodic ocular movements that cause subjective visual shimmering, ocular trembling, and/or tilted vision) Entrapment of the trochlear nerve by a vascular loop (similar to the pathophysiology [merckmanuals.com]

Pathophysiology [ edit ] The fourth cranial nerve innervates the superior oblique muscle for each eye. The superior oblique muscle is one of the six extraocular muscles that allow movement of eye. [en.wikipedia.org]

[…] adrenoleukodystrophy, 113 Neonatal facial asymmetry, 169-192 Neostigmine, 141 Nerve injuries, 266 Neuroaxonal dystrophy, 104 Neuroblastoma, 257 Neurocutaneous melanosis, 301 Neurocutaneous syndrome, 287, 295-308 Neurofibromatosis, 301 Neuromuscular disorders, pathophysiology [pediatricneuro.com]

Prevention

Other eye doctors recommend surgery early in a patient's life to prevent the compensatory torticollis and facial asymmetry that develop with age. [en.wikipedia.org]

Prevention Prevention of paralysis depends on prevention of the underlying causes. Risk of stroke can be reduced by controlling high blood pressure and cholesterol levels. [medical-dictionary.thefreedictionary.com]

It is unknown, however, whether early strabismus surgery can prevent or reverse facial asymmetry. [guwsmedical.info]

Therefore, extreme measures are taken in hospital settings to prevent the condition by reducing intracranial pressure, or decompressing (draining) a hematoma which is putting local pressure on a part of the brain. [howlingpixel.com]

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