Opsoclonus-Myoclonus Syndrome in Patients With Locked-in Syndrome: A Therapeutic Porthole With Gabapentin

Pistoia, Francesca; Conson, Massimiliano; Sarà, Marco
June 2010
Mayo Clinic Proceedings;Jun2010, Vol. 85 Issue 6, p527
Academic Journal
Patients with locked-in syndrome, although fully conscious, have quadriplegia, mutism, and lower cranial nerve paralysis. The preservation of vertical gaze and upper eyelid movements usually enables them to interact with the environment through an eye-coded communication. However, locked-in syndrome may be complicated by the development of an opsoclonus-myoclonus syndrome that may represent an additional impediment to communication. We evaluated whether off-label treatment with gabapentin could help patients with locked-in syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. A mechanism responsible for gabapentin-induced improvement has been also hypothesized. In this study, 4 patients presenting with locked-in syndrome complicated by opsoclonus-myoclonus syndrome were continuously treated with gabapentin up to 1200 mg/d. The treatment resulted in a rapid and long-lasting resolution of opsoclonus-myoclonus symptoms without adverse effects. After 2 weeks, patients showed voluntary attempts to communicate through eye blinking and thereafter regained voluntary control of full eye movements. This event enabled them to regain a communication channel with relatives and physicians and to start using eye-controlled brain-computer interfaces. Because of its effectiveness in restoring eye movement control, gabapentin opened a communicative porthole in the patients' lives. Since opsoclonus may be related to disorders of the inhibitory control of saccadic burst neurons by pontine pause cells, we hypothesize that gabapentin acts as a regulator of saccadic circuits.


Related Articles

  • Cranial Nerve Defects in Congenital Facial Palsy. Carr, Michele M.; Ross, David A.; Zuker, Ronald M. // Journal of Otolaryngology;Mar/Apr1997, Vol. 26 Issue 2, p80 

    Methods: Cranial nerve defects were enumerated in 29 children with congenital facial palsy presenting for reanimation via chart review with a view to determining unaffected donor nerves. The literature was searched via Medline to reveal 186 additional case reports that were complete enough in...

  • Weber's syndrome.  // Taber's Cyclopedic Medical Dictionary;2005, p2346 

    A definition of the term "Weber's syndrome" is presented. It refers to hypoglossal nerve paralysis on one side and oculomotor nerve paralysis on the other side accompanied with the paralysis of limbs due to a lesion of a cerebral peduncle.

  • A Young Boy with Painful Eye Swelling and Facial Rash. Mahbub, Md. Shahriar; Al Faruq, Abdullah; Rahman Siddiqui, Md. Mahmudur; Imran Khan, Mohammad Ashik; Ahasan, Ham Nazmul; Alam, Md. Billal; Miah, Md. Titu; Gupta, Ratan Das // Journal of Medicine;Jan2011, Vol. 12 Issue 1, p91 

    Oculomotor palsy is a recognized uncommon complication in herpes zoster ophthalmicus. A case of herpes zoster ophthalmicus with ophthalmoplegia is reported. A 15 year old boy presented with ptosis, chemosis, proptosis, ophthalmoplegia involving IVth and VIth cranial nerve following vesicular...

  • Childhood steroid-responsive ophthalmoplegic migraine. Roy, Mahua; Ghosh, Jagabandhu; Deb, Sujit; Pandit, Narayan // Journal of Pediatric Neurosciences;Jan-Jun2011, Vol. 6 Issue 1, p69 

    Ophthalmoplegic migraine (OM) is characterized by recurrent attacks of headache with paresis of ocular cranial nerves. Previously, it was classified as a variant of migraine, but recently, International Headache Classification (IHCD-II) has reclassified OM to the category of neuralgia....

  • Çocukluk Çağında üçncüSinir Felci Ä°le Seyreden AÄŸrılı Oftalmopleji. Ekinci Köktekir, Bengü; Gedik, Şansal; Bakbak, Berker // Turkish Journal of Ophthalmology / Turk Oftalmoloji Dergisi;Apr2012, Vol. 42 Issue 2, p159 

    The causes of painful ophthalmoplegia that manifests with third nerve palsy in childhood include postinfectious third nerve palsy, compressive lesions, vascular anomalies and ophthalmoplegic migraine. Ophthalmoplegic migraine is a rare pathology in the literature and is characterized by...

  • Postpartum pituitary apoplexy with isolated oculomotor nerve palsy: A rare medical emergency. Raina, Sujeet; Jearth, Vaneet; Sharma, Ashish; Sharma, Rajesh; Mistry, Kewal; Roerink, S. H. P. P. // Journal of Neurosciences in Rural Practice;Oct-Dec2015, Vol. 6 Issue 4, p598 

    Pituitary apoplexy is a clinical syndrome characterized by sudden onset headache, visual deficits, ophthalmoplegia, altered mental status, and hormonal dysfunction due to an expanding mass within the sella turcica resulting from hemorrhage or infarction of pituitary gland. We report a case of...

  • Neuroimaging of isolated and non-isolated third nerve palsies. Lo, C.-P.; Huang, C.-F.; Hsu, C.-C.; Kuo, C.-C.; Liu, C.-C.; Wang, Y.-M.; Wang, W.-Y. // British Journal of Radiology;Apr2012, Vol. 85 Issue 1012, p460 

    A variety of aetiologies may cause third nerve palsy (TNP), and some manifestations may herald neurological emergencies. This article describes and illustrates various diseases that lead to TNP.

  • Cranial nerves supplying the extraocular muscles. Grant, Simon // Optometry Today;7/30/2010, Vol. 50 Issue 15, p34 

    The article examines the anatomy of cranial nerves, their potential causes of damage and diagnosis of ocular motor palsies. The general features of cranial nerves are oculomotor nuclei, trochlear nuclei and abducens nuclei. A discussion of the 3rd cranial nerve which traverses the medial...

  • Recurrent Functional Pituitary Adenoma Presenting as Oculomotor Nerve Infiltration: Case Report. Gülsen, Salih; Yilmaz, Cem; Caner, Hakan; AltinÖrs, Nur // Medical Journal of Trakya University / Trakya Universitesi Tip F;2009, Vol. 26 Issue 3, p277 

    It is estimated that pituitary tumors account for 10% to 15% of all primary brain tumors. Patients with pituitary adenoma and cranial nerve involvement should be evaluated regarding possible oculomotor nerve infiltration. We present this case to emphasize the importance of diagnosing...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics