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Congenital Abducens Nerve Palsy

Congenital Sixth Cranial Nerve Palsy


Presentation

  • The signs and symptoms of neurological disease are elusive by their very nature, presenting a confounding diagnostic challenge.[books.google.com]
  • If the child presents soon after onset, suppression of the non-dominant eye has usually not been present long enough to produce suppression amblyopia, but a difference in visual acuity may be present later on.[reviewofophthalmology.com]
  • Some patients may also present with this characteristic ocular motility pattern without overt craniofacial signs ( Figure 1 ).[nature.com]
Falling
  • These children continue to "fall through the cracks" of our medical education system.[books.google.com]
  • Epidemiology Frequency United StatesSixth nerve palsies fall into the following categories: 8-30% idiopathic, 10-30% miscellaneous, 3-30% trauma, 0-6% aneurysm, and 0-36% ischemic.[thehealthscience.com]
  • […] brain that occurs at or shortly after birth) metabolic disorder (a disorder that interferes with the body's ability to maintain itself) Damage to the spinal cord is most often caused by trauma, such as a fall or a car crash.[medical-dictionary.thefreedictionary.com]
  • Binocular Vision & Strabismus Quarterly. 13(3 3rd Qtr):177-80, 1998 Fall. Surgical treatment of superior oblique palsy. Transactions of the American Ophthalmological Society. 94:315-28; discussion 328-34, 1996.[www2.medicine.mcgill.ca]
  • Bemerkungen zu einem Fall von Retraktion des Auges. Centralbl Pract Augenheilkd. 1899;23:14. Google Scholar 16. Hoyt WF, Nachtigaller H.[link.springer.com]
Cat Scratch
  • Trauma and vascular disease are considered the main causes of acquired CN IV palsy. 14,15 However, numerous reports of other potential causes of isolated CN IV palsy, include multiple sclerosis, polycythema vera, cat-scratch disease and, rarely, metastatic[reviewofoptometry.com]
  • Trauma and vascular disease are considered the main causes of acquired CN IV palsy. 14,15 However, numerous reports of other potential, but less common, causes of isolated CN IV palsy exist, including multiple sclerosis, polycythema vera, cat-scratch[reviewdiseasehandbook.com]
Hoarseness
  • Etiology Clinical features Loss of the gag reflex (efferent limb) Flaccid paralysis of the soft palate nasal speech and deviation of the uvula away from the lesion Epiglottic paralysis aspiration Dysphagia Features of vocal cord paralysis Dysphonia (hoarseness[amboss.com]
Strabismus
  • New Fifth Edition chapters cover Neonatal Ophthalmology, Surgical Management of Strabismus, Medicolegal Issues, and Evolution of Strabismus Surgery.[books.google.com]
  • Materials and Methods: A retrospective review of strabismus patient charts was performed and paralytic strabismus was isolated and evaluated for age, sex, etiology, type of strabismus and lateralization.[austinpublishinggroup.com]
  • Pathophysiology of strabismus Strabismus often occurs in children who are otherwise completely normal.[patient.info]
Torticollis
  • Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis.[books.google.com]
  • Infants noted to have torticollis, or a sustained head tilt, may be referred to a pediatric ophthalmologist to be evaluated for a congenital fourth nerve palsy. Fourth nerve palsies can also be caused by head trauma, infection, or a brain mass.[willseye.org]
  • Amblyopia of either eye may occur if the patient does not have binocular vision and does not maintain torticollis. Figure 19-1.[entokey.com]
  • 弯足 coccyx 尾骨 cold abscess冷膿瘍 collagen disease膠原病(こうげんびょう) collateral ligament側副靭帯 compress1.湿布、2.圧迫包帯 compression fracture圧迫骨折 computed tomography (CT) コンピュータ断層撮影[法] condyle顆(か) congenital dislocation of the hip (CDH)先天性股関節脱臼、先天股脱 congenital muscular torticollis[tokyo-med.ac.jp]
Muscle Swelling
  • DIFFERENTIAL DIAGNOSIS WHY Duane’s retraction syndrome Difficulty on ABD & ADD with eyelid retraction Grave’s orbitopathy (TED) Proptosis decreased ability of eye movement diplopia Orbital trauma Orbital fracture Muscle swelling eye restriction ABD &[slideshare.net]
Nystagmus
  • Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis.[books.google.com]
  • MRI is indicated for any brainstem findings to exclude pontine glioma in children (most have papilledema and nystagmus without other cranial nerve involvement) and in adults who show no improvement.[emedicine.medscape.com]
  • (Reisner et al. 1971) • May represent one of following: Duane syndrome, infantile esotropia, nystagmus blockage syndrome (Ansons & Davis, 2014) 16.[slideshare.net]
Steppage Gait
  • gait鶏歩行、鶏歩き、鶏歩 stiffness1.硬直、こわばり、2.不撓性〈脊椎の〉、3.剛性 straight leg raising test (SLR test)下肢伸展挙上テスト stress fracture疲労骨折 subarachnoid spaceくも膜下腔 subclavian artery鎖骨下動脈 Sudeck's atrophy Sudeck骨萎縮 suppository坐剤 supraclavicular fossa鎖骨上窩(―じょうか) suture1.縫合[法][tokyo-med.ac.jp]
Long Tract Signs
  • tract signs (weakness), as well as the possible involvement of cranial nerves V, VII, and VIII ( Table 1 ).[arabmedmag.com]
Nasal Speech
  • Vagus nerve lesion (X) Etiology Clinical features Loss of the gag reflex (efferent limb) Flaccid paralysis of the soft palate nasal speech and deviation of the uvula away from the lesion Epiglottic paralysis aspiration Dysphagia Features of vocal cord[amboss.com]
Neck Stiffness
  • stiffness Additional symptoms depend on which cranial nerves are involved.[amboss.com]

Workup

  • Mortality/Morbidity A young patient should have an aggressive workup because of the greater likelihood of a neoplasm causing the palsy.[thehealthscience.com]
  • Testing: A suggested workup for 6th nerve palsy, adapted from Galetta and Lawton Smith (1989) is as follows: At presentation: General physical, ophthalmologic and neurologic Blood pressure Ascultation for bruits over the eyes and mastoid Cocaine test[dizziness-and-balance.com]
  • Non-traumatic third nerve palsy cases must undergo a full workups with neuro-imaging. The usual clinical sign of a third nerve palsy is the outward and downward location of the involved eye. This may or may not be accompanied by lid drooping.[wrighteyecare.com]
  • For any patient who develops signs of aberrant regeneration, workup for a structural lesion is initiated, or repeated if previous workup has been carried out and no lesion found. Nonisolated Cranial Neuropathies MULTIPLE CRANIAL NEUROPATHIES.[medtextfree.wordpress.com]
  • Natural History and Clinical Workup Newborns may demonstrate a transient sixth nerve palsy that is frequently unilateral and occasionally accompanied by a temporary ipsilateral seventh nerve palsy.53,267,291,400 Simple observa- FIGURE 5-7.[guwsmedical.info]
Hypercholesterolemia
  • Some risk factors include multiple sclerosis, encephalitis, meningitis, cavernous sinus thrombosis, hypertension, hypercholesterolemia, aneurysm, diabetes, arteriosclerosis, and birth trauma.[eyewiki.aao.org]

Treatment

  • Thorough coverage for each neurological disease clearly defines age at onset, course of illness, clinical features, and treatment options. Differential diagnosis tables and treatment algorithms expedite clinical decision making.[books.google.com]
  • Non-surgical methods of treatment must be used temporarily during this period to eliminate the troublesome symptom of diplopia.[en.wikiversity.org]
  • It can be used as the sole treatment for sixth nerve palsy, or in addition to a surgical treatment. The operations performed in this study were either transpositions or recess-resect procedures.[healio.com]

Prognosis

  • Palsies related to trauma or brain masses have a guarded prognosis and recovery, if any, may take up to one year.[encyclopedia.com]
  • Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol. 1992 May;113(5):489-96. 16. Bagheri A, Eshaghi M.[reviewofoptometry.com]
  •  Introduction  Anatomy  Sixth NervePalsy o Incidence o Etiology o Differential Diagnosis o Clinical Signs& Symptoms o Optometric Assessment & Investigation o Other Clinical Assessment & Investigation o Optometric Management o Other Managements o Prognosis[slideshare.net]
  • Cause and prognosis in 1,000 cases.Arch Ophthalmol 1981 Jan; 99(1): 76-9 [Medline]. von Noorden GK, Murray E, Wong SY: Superior Oblique Paralysis.Arch Ophthalmol 1986; 104: 1771-1776.[www2.medicine.mcgill.ca]
  • […] 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]

Etiology

  • The aim of this study is to describe demographics and etiologic factors of patients with paralytic strabismus.[austinpublishinggroup.com]
  • Conversely, slowly progressive onset suggests a compressive etiology. Subacute onset suggests a demyelinating process as a possible etiology. Associated pain suggests a microvascular etiology.[eyewiki.aao.org]
  • An inflammatory etiology, such as Tolosa-Hunt syndrome, is painful and typically responds quickly to steroids. Other etiologic considerations would include aneurysm, bone metastasis, and ophthalmoplegic migraine.[arabmedmag.com]
  • Etiology Clinical features Extorsion of the eye : inability to depress and adduct the eyeball simultaneously (the pupil shoots upward during attempted adduction of the eyeball) Diplopia ( double vision ) Mild esotropia Trigeminal nerve lesion (V) Etiology[amboss.com]
  • Etiology of Infranuclear Sixth Nerve Palsy.[guwsmedical.info]

Epidemiology

  • […] 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 United StatesSixth nerve palsies fall into the following categories: 8-30% idiopathic, 10-30% miscellaneous, 3-30% trauma, 0-6% aneurysm, and 0-36% ischemic.[thehealthscience.com]
  • "[Epidemiological and clinical aspects of Stilling-Turk-Duane syndrome]". J. Fr. Ophtalmol. (in French). 19 (8–9): 533–42. PMID 8944136. Further reading [ edit ] Andrews, Caroline V.; Hunter, David G.; Engle, Elizabeth C. (1993). "Duane Syndrome".[en.wikipedia.org]
  • This study was performed to determine epidemiologic and etiologic distribution and results of strabismus surgery in patients with third nerve palsy referred to our clinic as a tertiary center over a 10-year period.[jovr.org]
  • Epidemiology In 2007, the Moebius Syndrome Foundation estimated that there were at the time a total of approximately 2,000 cases of Moebius Syndrome worldwide [6].[physio-pedia.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Only the ipsilateral lateral rectus that is solely innervated by the involved peripheral sixth cranial nerve is affected; therefore, only deviations in the horizontal plane are produced.[thehealthscience.com]
  • Pathophysiology of strabismus Strabismus often occurs in children who are otherwise completely normal.[patient.info]
  • Pathophysiology The fourth cranial nerve nucleus is located in the dorsal mesencephalon. Nerve fibers decussate and exit the brain stem dorsally into the subarachnoid space.[reviewdiseasehandbook.com]
  • Cadera et al 24 studied pathophysiology of double elevator palsy in two patients with magnetic resonance imaging (MRI) with volume scanning technique.[entokey.com]

Prevention

  • The hope is that preventing secondary contracture of the medial rectus muscle will increase the chances of recovery without strabismus surgery.[wrighteyecare.com]
  • Therefore, if close follow-up to resolution of the palsy or paresis is not possible, neuroimaging is recommended.24 Treatment Amblyopia prevention is always key in children younger than 7 to 9 years of age.[guwsmedical.info]
  • 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]
  • Occlusion using Bangerter filter or pirate patch can eliminate diplopia and confusion, prevent amblyopia or suppression in younger patients, and decrease the possibility of ipsilateral medial rectus contracture.[eyewiki.aao.org]
  • Babies require the use of artificial tears during their waking hours, and eye ointments during sleep, to prevent eye dryness because of inability to close the eyelids completely.[news-medical.net]

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