Millard-Gubler syndrome is a rare lesion of the brainstem affecting the abducens (VI) and the facial (VII) cranial nerves, as well as the pyramidal tract. Unilateral palsy of these nerves accompanied by contralateral motor weakness of the limbs is the typical clinical presentation. The diagnosis is made on clinical grounds, and the underlying cause often requires a thorough imaging workup.
Presentation
Millard-Gubler syndrome, initially described more than 150 years ago, is a lesion of the brainstem that develops in the ventral pons, and the term "ventral pontine syndrome" is often used as a synonym [1] [2] [3]. More specifically, it belongs to a group of "crossed paralysis syndrome", as it is responsible for causing facial lesions on one side and limb deficits on the other [1] [2]. Scarce reports exist regarding its etiology, but vascular insults, tumors, and infectious diseases (neurocysticercosis and tuberculosis) have been described [1] [3] [4] [5]. The clinical presentation is distinguished by unilateral palsy of the cranial nerves VI and VII (abducens and facial nerves, respectively), together with contralateral hemiparesis, as the corticospinal tract is affected before its decussation [4] [5] [6]. Hence, weakness of the lateral rectus muscle will lead to diplopia and medial deviation of the affected eye, whereas lesions of the facial nerve (responsible for innervating the muscles of facial expression, including the platysma, orbicularis oculi, mentalis, orbicularis oris, and several other), will present with deviation of the mouth angle and a variable degree of facial paralysis [1] [2] [5]. Millard-Gubler syndrome is distinguished from other similar brainstem disorders by the absence of sensory deficits, as both the medial lemniscus and the spinothalamic tracts are not damaged [1].
Entire Body System
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Painter
Gubler Adolphe-Marie Gubler (1821 – 1879), French physician and pharmacologist Millard–Gubler syndrome Eduard Gubler (1891–1971), Swiss painter Matthew Gray Gubler (born March 9, 1980), American actor and former fashion model Max Gubler (1898 - 1973), [howlingpixel.com]
Gastrointestinal
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Regurgitation
There was no history of fever, headache, seizures, altered sensorium, diplopia, nasal regurgitation of feeds, trauma, ear discharge, rash, recent vaccination, and history of contact with an open or treated case of tuberculosis. [ruralneuropractice.com]
Eyes
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Diplopia
Thus the diplopia is horizontal and worse in the distance. [howlingpixel.com]
[…] crossed paralysis syndromes, which are characterized by cranial nerves VI and VII palsies with contralateral body motor or sensory disturbances 1-3. ipsilateral facial and contralateral body hemiplegia due to pyramidal tract involvement 1-3 isotropism and diplopia [radiopaedia.org]
Symptoms include: Contralateral hemiplegia (sparing the face) due to pyramidal tract involvement Ipsilateral lateral rectus palsy with diplopia that is accentuated when the patient looks toward the lesion, due to cranial nerve VI involvement. [strokecenter.org]
Paralysis of the abducens (CN VI) leads to diplopia, internal strabismus (i.e., esotropia), and loss of power to rotate the affected eye outward), and disruption of the facial nerves (CN VII) leads to symptoms including flaccid paralysis of the muscles [operativeneurosurgery.com]
Paralysis of the abducens (CN VI) leads to diplopia, internal strabismus, and loss of power to rotate the affected eye outward), and disruption of the facial nerves (CN VII) leads to symptoms including flaccid paralysis of the muscles of facial expression [mnemonics-usmle.blogspot.com]
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Retinal Cyst
On fundus examination, there was no retinal cyst and evidence of papilloedema. She was oriented and hemodynamically stable. Rest of the systemic examination was unremarkable. [ruralneuropractice.com]
Neurologic
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Long Tract Signs
Therefore, the clinical signs of the cranial nerve are ipsilateral to the lesion, and the long tract signs contralateral, resulting in crossed syndrome. [ncbi.nlm.nih.gov]
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Expressive Aphasia
aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect Gerstmann syndrome Astereognosis occipital lobe : Bálint's syndrome Cortical blindness Pure alexia temporal lobe : Cortical deafness Prosopagnosia Thalamus Thalamic syndrome Other [en.wikipedia.org]
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Cranial Nerve Involvement
Nerves Involved Parinaud Syndrome Tracts Involved Supranuclear mechanism for forward upward gaze and periaquaductal gray matter Paralysis of upward gaze and accommodation, fixed pupils Pinealoma and other lesions of dorsal midbrain, hydrocephalus Millard-Gubler [quizlet.com]
Workup
The diagnosis of Millard-Gubler syndrome rests on the ability of the physician to recognize this rare brainstem syndrome, which can be done only by performing a detailed physical examination. Lesions of both abducens and facial nerve, as well as hemiplegia, must be confirmed by a properly conducted neurological exam. Because of the fact that different etiologies might induce Millard-Gubler syndrome, a complete patient history should be taken as well, in order to identify whether additional symptoms could possibly point toward an infection (fever, for example) or a vascular insult (presence of additional neurological deficits). Regardless, the use of imaging studies should be advised, and computed tomography (CT), but more commonly magnetic resonance imaging (MRI), are effective methods for visualizing the ventral aspect of the pons [1] [4] [5]. More importantly, the associated lesions (tuberculomas, tumors, or cysticercus granulomas) can be readily identified using these methods [3] [5].
Microbiology
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Taenia Solium
In North America, the largest number of neurosurgical cases stemming from parasitic infections involves the larval form of Taenia solium, the infectious organism causing neurocysticercosis. [scienceopen.com]
J Neurosci Rural Pract 2012;3:375-7 Neurocysticercosis is the most common parasitic infection of central nervous system and result from infection with the intermediate stage of Taenia solium. [ruralneuropractice.com]
Treatment
Efficacy of treatment should be monitored by repeat CT/MRI after 3 months. [7] Our patient improved and became asymptomatic with steroid treatment and albendazole. [ruralneuropractice.com]
Endovascular treatment for intracranial vertebrobasilar artery stenosis. Acta Neurochir Suppl. 2014. 119:83-9. [Medline]. Alexander MD, Rebhun JM, Hetts SW, et al. [medscape.com]
Treatment / Management Treatment mainly depends on the etiology of the disease. In some cases, patients presenting with multiple deficits require early conservative measures together with multidisciplinary rehabilitation. [ncbi.nlm.nih.gov]
Prognosis
Prognosis Prognosis mainly depends on the extent and etiology of the disease. A vertebrobasilar stroke usually leaves a significant neurologic deficit. Patients with small lesions usually have a better prognosis. [ncbi.nlm.nih.gov]
Etiology
Etiology Causes of Millard-Gubler syndrome (MGS) vary with age. [ncbi.nlm.nih.gov]
Epidemiology
in patients who have one major plus two minor criteria, in those who have one major plus one minor and one epidemiologic criterion, and in those who have three minor plus one epidemiologic criterion. [scienceopen.com]
The book also provides updated epidemiologic and statistical data throughout and includes a section on biostatistics in physical medicine and rehabilitation. [books.google.com]
Epidemiology Epidemiology varies according to the cause of the disease. Pathophysiology This syndrome is caused due to a lesion at ventral part of the pons that involves the fibers of cranial nerves VI, VII, and corticospinal tract fibers. [ncbi.nlm.nih.gov]
Pathophysiology
[…] signification worked out study than one exclusively devoted to a of imaging and magnetic resonance imaging, which limited number of scientific papers. will perhaps allow us to detect ischemic edema in the After a brief clinical and pathophysiological [books.google.com]
Pathophysiology This syndrome is caused due to a lesion at ventral part of the pons that involves the fibers of cranial nerves VI, VII, and corticospinal tract fibers. [ncbi.nlm.nih.gov]
Prevention
Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. [books.google.es]
Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Actigraphy Parasomnia Disease mongering v t e Pathology of the nervous system, primarily CNS ( G00-G47, 320-349 ) Inflammatory diseases of the [wikidoc.org]
Even with vertebral artery occlusion, collaterals (circle of Willis) may prevent ischaemia. [patient.info]
Ximelagatran vs warfarin for stroke prevention in patients with nonvalvular atrial fibrillation: a randomized trial. JAMA. 2005 Feb 9. 293(6):690-8. [Medline]. [Full Text]. Kim YW, Hong JM, Park DG, et al. [medscape.com]
Prevention of recurrent stroke Strict risk factor control is important to decrease the risk of stroke recurrence. [41] Prevention strategies depend on the primary cause of the stroke. [emedicine.medscape.com]
References
- Matlis A, Kleinman Y, Korn-Lubetzki I. Millard-Gubler syndrome. AJNR Am J Neuroradiol. 1994;15(1):179-181.
- Kesikburun S, Safaz I, Alaca R. Pontine cavernoma hemorrhage leading to Millard-Gubler syndrome. Am J Phys Med Rehabil. 2011;90(3):263.
- Prasad R, Kapoor K, Srivastava A, Mishra O. Neurocysticercosis presenting as Millard Gubler syndrome. J Neurosci Rural Pract. 2012;3(3):375-377.
- Yasuda Y, Matsuda I, Sakagami T, Kobayashi H, Kameyama M. Pontine infarction with pure Millard-Gubler syndrome: precise localization with magnetic resonance imaging. Eur Neurol. 1993;33(4):331-334.
- Sharif M, More V, Purandare S. Brainstem tuberculoma--presenting as Millard Gublar syndrome. Indian J Pediatr. 2010;77(6):707.
- Azarmina M, Azarmina H. The Six Syndromes of the Sixth Cranial Nerve. J Ophthalmic Vis Res. 2013;8(2):160-171.