A 64-year-old woman, diabetic and hypertensive, presented with sudden onset painless diplopia and alteration of consciousness. She was drowsy with neurological examination revealing exotropia of both eyes in primary position with bilateral internuclear ophthalmoplegia on attempted horizontal gaze (Figure 1a). Pupils were normal bilaterally. Convergence, vertical saccades, pursuits and vestibula...

BACKGROUND Niemann-Pick Type C disease (NPC), is an autosomal recessive neurovisceral disorder of lipid metabolism. One characteristic feature of NPC is a vertical supranuclear gaze palsy particularly affecting saccades. However, horizontal saccades are also impaired and as a consequence a parameter related to horizontal peak saccadic velocity was used as an outcome measure in the clinical tria...

Examination of the pupillary response to light and accommodation can provide precise information concerning the localization of a lesion within the sympathetic and parasympathetic pathways, the anterior visual pathway, or the brainstem (Table 1). Classical teaching has associated all lesions of the rostral midbrain with bilateral lightnear dissociation (LND), defined as attenuation of the pupil...

A 62-year-old woman with relapsing-remitting multiple sclerosis suddenly complained of diplopia associated with bilateral adduction impairment, nystagmus of the abducting eye bilaterally, and sparing of abduction, convergence, and vertical eye movements, consistent with bilateral internuclear ophthalmoplegia. Within 1 week, she had developed a complete horizontal gaze paralysis even with the oc...

A 50-year-old man was admitted for the acute onset of vertical diplopia worse on left gaze. The right eye appeared hypertropic. Either right fourth nerve palsy or skew deviation was possible.1,2 Nonmydriatic retinal photography revealed an excyclotorsion of the hypotropic eye associated with incyclotorsion of the hypertropic eye (figure 1, A and B) providing a key element for skew deviation. In...

Progressive supranuclear palsy (PSP) is an uncommon disorder characterized by marked postural instability, vertical gaze abnormalities and axial rigidity. The purpose of this study is to report the clinical features of 16 consecutive subjects seen over a 10-year period at a Movement Disorders Clinic. These subjects fulfilled criteria for probable PSP namely those of the National Institute of Ne...

J.S. Kim, MD S.-H. Jeong, MD Y.-M. Oh, MD Y. Soon Yang, MD S.Y. Kim, MD A 78-year-old man with hypertension and diabetes was referred because of 3 days of diplopia and ophthalmoplegia. Neurologic examination disclosed exotropia of both eyes (wall-eyed) and bilateral internuclear ophthalmoplegia (WEBINO) with impaired convergence (figure, A).1 Vertical saccades and smooth pursuit were also limit...

Niemann-Pick type C disease (NPC) is a rare lysosomal disease with a protean presentation, ranging from a fatal neonatal course with visceromegaly to an adult presentation with only neurological or psychiatric symptomatology. In this report we describe the genetic and clinical characteristics of 3 Mexican patients from different families with juvenile presentation of NPC. Clinical examination, ...