Paroxysmal Tonic Upgaze Presenting as a Clinical Isolated Syndrome

Abstract

We report a 3.5-year-old boy with sudden onset of episodic upward deviation of the eyes that led to diagnosis of paroxysmal tonic upgaze. Cranial magnetic resonance imaging showed right-hand side dominant bilateral hyperintense lesions in the mesencephalon and the thalamus on T2-weighted images. These lesions suggested a demyelinating pathology. Corticosteroid treatment was started. The episodes of upward eye deviation and the lesions on magnetic resonance imaging completely resolved after 3 days and 3 months, respectively. After 2 years of follow-up, he was symptom-free. Our patient with an isolated brain stem syndrome and no mental status changes was diagnosed as having a clinical isolated syndrome.

Keywords

paroxysmal tonic upgaze clinical isolated syndrome acute disseminated encephalomyelitis

Get full access to this article

View all access options for this article.

References

Ouvrier R. , Billson F. Paroxysmal tonic upgaze of childhood-a review. Brain Dev. 2005;27:185-188.

Blumkin L. , Lev D. , Watemberg N. , Lerman-Sagie T. Hypomyelinating leukoencephalopathy with paroysmal tonic upgaze and absence of psychomotor development. Mov Disord. 2007 ;2: 226-228.

Hayman M. , Harvey AS , Hopkins IJ , Kornbeg AJ , Coleman LT , Shield LK Paroxysmal tonic upgaze: a reappraisal of outcome. Ann Neurol . 1998;43:514-520.

Rasmussen M. , Kerty E. Benign paroxysmal tonic upgaze of a child with positive MRI findings. J Pediatr Ophthalmol Strabismus . 1999;36:164-166.

Spalice A. , Parisi P. , Ianetti P. Paroxysmal tonic upgaze: pathophysiological considerations in three additional cases. J Child Neurol. 2000;15:15-18.

Sugie H. , Sugie Y. , Ito M. , Tsurusi S. , et al. A case of paroxysmal tonic upward gaze associated with psychomotor retardation. Dev Med Child Neurol. 1995;37:362-365.

Lavin PJM , Donahue SP Neuro-ophthalmology: ocular motor system . In: Bradley WG , ed. Neurology in Clinical Practice. Oxford, UK: Butterworth-Heinemann; 2000:699-720.

Tenenbaum S. , Chitnis T. , Ness J. , Hahn JS , and the International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology. 2007;68(suppl 2):S23-S36.

Fırat AK , Karakaş HM , Yakıncı C. , Altınok T. , Alkan A. , Bıçak U. An unusual case of acute disseminated encephalomyelitis confined to brainstem . Magn Reson Imaging. 2004;22: 1329-1332.

10.

Miller DH , Scaravilli F. , Thomas DC , Harvey P. , Hirsch NP Acute disseminated encephalomyelitis presenting as a solitary brainstem mass . J Neurol Neurosurg Psychiatry. 1993 ;56: 920-922.