A 72-year-old man with double vision
Digital Journal of Ophthalmology 2007
Volume 13, Number 7
February 17, 2007
Printer Friendly



Sreekumari Pushpoth | Wolverhampton and Midlands Eye Infirmary
Stephanie Smith | East Lancashire Hospitals NHS Trust
J.P. Roper | East Lancashire Hospitals NHS Trust


History
A 72 year-old gentleman was referred to ophthalmology department with complaints of occasional intermittent binocular double vision for the past eight months. The patient did not complain of any headache, jaw claudication, weight loss, diurnal variation of symptoms or breathing difficulty. On inquiring about general health, the caretaker of the patient said that he had become increasingly forgetful and had a history of recurrent falls. There was no past medical history of hypertension or diabetes mellitus.

Examination
On examination, visual acuity was 6/9 in both eyes without correction. There was no proptosis, with a Hertel’s exophthalmometry reading of 18 mm in either eye. There was 25 prism diopter exotropia present at 1/3 meter and 6 meters. There was no movement on elevation or depression (duction or version). The eye movement was slow on horizontal gaze, but normal. Convergence was absent. Dolls eye movement was normal. Vertical saccades were absent, horizontal saccades slow. There was no response to optokinetic nystagmus drum. Anterior segment evaluation was unremarkable with equal size of pupils, briskly reacting to direct and consensual reflex. Fundus evaluation was normal. General examination showed lack of expression with axial rigidity and shuffled gait. There was no resting tremor.

Figure 1
 Attempted upgaze

Figure 2
 Attempted down gaze

Figure 3
 Right gaze

Figure 4
 Left gaze

Ancillary Testing
Radiographic Studies
CT scan showed generalized cortical atrophy.

Treatment
As the patient’s symptoms were related to his general condition, he was referred to his primary physician for further care.

Differential Diagnosis
Progressive Supra Nuclear Gaze Palsy
Whipple’s Disease
Diffuse Lewy body disease
Myasthenia Gravis
Thyroid eye disease

Diagnosis and Discussion
Progressive Supra Nuclear Gaze Palsy

Progressive supranuclear palsy (PSP), (also known as Nuchal Dystonia Dementia Syndrome, Steele-Richardson-Olszewski Syndrome) is an important, often under-diagnosed neurodegenerative syndrome, which affects the brainstem and basal ganglia. It was first described as a distinct syndrome by John Steele, J Clifford Richardson and Jerzy Olszewski in 1963.


Patients usually present with postural instability with frequent falls. In the earliest stages of the disease there may be slowness of vertical saccadic eye movements which progress to limitation of downwards vertical saccadic eye movements and then to a complete vertical gaze palsy.(1) The doll's head maneuver demonstrates a normal vertical vestibular-ocular response. This confirms the integrity of the third nerve nuclei and places the pathology of the eye movement at supranuclear level.(2) Over activity of the frontalis muscle combined with diminished blink rate lead to a “surprised” facial appearance. Eye opening may be impaired either by active involuntary contraction of orbicularis oculi (blepharospasm) or by inability to voluntarily open the eyes (“apraxia of eyelid opening”).(3) Patients may also have cognitive dysfunction, axial rigidity, dysphagia and dysarthria.

Patients usually present with symptoms between 50 and 77 years of age.
The disease process is persistently progressive. Supportive treatment is the mainstay of management. Physiotherapy and occupational therapy are important in helping with aids for balance and avoidance of falls. Another difficulty faced by these patients is with swallowing. Speech therapy services have an important role in swallowing assessment, mainly to avoid the complications of aspiration.(4)

The prevalence of PSP is often underestimated as it is often misdiagnosed as Parkinson’s Disease (PD).(5, 6) It is very important to differentiate between the two, as patients with PSP need more supportive therapy. Features like taut spastic face, dysarthria, neck held in extension with axial rigidity, a symmetrical mild distal bradykinesia, normal muscle tone and absence of rest tremor helps to clinically differentiate PSP from PD.

Better understanding of the disease process and its pathology may help in the identification of newer disease modifying agents for these patients.

References
1. Todd Troost BT, Daroff RB. The ocular motor defects in progressive supranuclear palsy. Ann Neurol 1977;2:397-403.

2. Huw R Morris, Nicholas W Wood, Andrew J Lees Progressive supranuclear palsy (Steele-Richardson-Olszewski disease) Postgrad Med J 1999;75:579-584.

3. Golbe LI, Davis PH, Lepore FE. Eyelid movement abnormalities in progressive supranuclear palsy. Mov Disord 1989; 4:297-302.

4. Litvan I, Sastry N, Sonies BC. Characterizing swallowing abnormalities in progressive supranuclear palsy. Neurology 1997;48:1654-1662.

5. Golbe LI. The epidemiology of PSP. J Neural Transm 1994; 42(suppl):263-273.

6. Hughes AJ, Daniel SE, Kilford L, Lees AJ. Accuracy of clinical diagnosis of idiopathic Parkinson's disease: a clinico-pathological study of 100 cases. J Neurol Neurosurg Psychiatry 1992;55:181-184.