A Korean medical journal recently published a neat little article on a single patient with the clinical diagnosis of PSP. Photos were taken of this gentleman over a four-year period (age 62, age 62, and age 66). There’s also a photo of this gentleman’s eyes when he was in his 40s. The photos show his eyes before the onset of ocular symptoms and after. Some MRI images are included as well. The full article is available for free online. I’ve copied the abstract and some excerpts below.
Journal of Korean Medical Science. 2009 Oct;24(5):982-4. Epub 2009 Sep 24.
Exodeviated ophthalmoplegia in a patient with progressive supranuclear palsy.
Kim C, Lee HW, Park MY.
Department of Neurology, Han Family Hospital, Daegu, Korea.
We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell’s phenomenon, doll’s eye movements, and vestibulo-ocular reflexes. These signs indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only ‘supranuclear’ but also ‘nuclear’ complete ophthalmoplegia with exodeviation of the eyes.
PubMed ID#: 19795006
Here’s a link to the full article (available online for free) and some excerpts:
“Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by progressive dystonic axial rigidity, postural instability, pseudobulbar palsy, subcortical dementia, and impaired of voluntary eye movements. Facial appearances are characteristic in PSP. The patients usually show a fixed ‘surprised’ stare, retracted head, raised eyebrows, markedly decreased blinks (less than 4 per min), and progressive downward gaze palsy. Previously, only a few reports describe PSP patients with photographs. We report a probable PSP patient with progressive eyeball deviations which was documented with serial photographs.”
“A 66-yr-old man was admitted because of dysphagia with repeated aspirations in March 2007. In October 2003, he was evaluated in a hospital because of blurred vision, dislike of bright lights and easy falls. He remembered several episodes of falling while riding a motorcycle since 2000. These episodes were not associated with feelings of dizziness or faintness and were independent of specific situation or body position. He visited several local eye clinics where he was diagnosed with bilateral cataracts and presbyopia, and underwent bilateral lens implantation in 2000. He did not benefit from the surgery, but felt a progressive worsening toward imbalance and unsteadiness when walking. Eye evaluation, in October 2003, revealed bilateral surgical pseudophakias and moderate exodeviation. Pupils were irregularly shaped and direct light reflexes were sluggish bilaterally. Fundoscopic examinations showed mildly increased cup to disk ratio (0.6 OD and 0.7 OS) with peripapillary atrophy in both eyes. Vertical ocular movements for willed gaze as well as following objects were slow and limited, though full ocular movements were elicited during passive head motions. Volitional horizontal gaze and pursuit of a visual target were also slow with bilateral adduction paresis. However, convergence was normal. Goldmann visual fields were also normal. He was slow and made errors when performing distal rapid alternating movements. He tended to topple backwards. There was neither resting nor action tremor. He was diagnosed with ‘parkinsonism’ and treated with levodopa/benserazide and other dopamine agonists, but his symptoms did not improve. His illness progressively worsened thereafter. He experienced frequent falls and needed a cane to walk. His head was retracted, and his voice was reduced to a slurred growl. He became unable to look down and had difficulties in swallowing. In November 2006 and January 2007, he suffered from aspiration pneumonias, and underwent tracheostomy and gastrostomy. Magnetic resonance imaging of the brain demonstrated moderate atrophy of the whole cerebrum and brainstem, as well as mild bihippocampal atrophy.”
“He was admitted to our hospital in March 2007. His medical history was otherwise unremarkable. His previous medications included levodopa/carbidopa, ropinirole, selegiline, and afloqualone. His family history was negative for gait, cognitive, or other neurological disorders. He responded correctly to simple verbal commands. His eyes deviated up and lateral, and his vertical and medial eye movements were absent. When the patient attempted to look laterally, only brisk quick lateral eyeball movements were observed on the same side, but the opposite eye showed no movement at all medially. He could not converge his eyes. There was neither Bell’s phenomenon nor optokinetic nystagmus. Caloric stimulations with both warm and cold water did not evoke any nystagmus bilaterally. Doll’s eye maneuver did not evoke any eyeball movements at all. However, because of the patient’s severe nuchal rigidity and retrocollis, the examination was suboptimal. He could not sit in bed by himself, and he repeatedly fell backward without support. His axial rigidity made his distal limb movements appear ataxic but when reaching for close objects with his back supported, his ataxic movements decreased markedly. Reflexes were symmetrical and minimally brisk in the upper and lower extremities with bilateral flexor plantar responses. There was no resting tremor.”
“Among the earliest signs of PSP is supranuclear gaze palsy. It includes slowing of voluntary downward saccades, a high percentage of errors in the antisaccade task, and frequent presence of square-wave jerks, and progression to a complete vertical gaze palsy. The doll’s head maneuver may generate a normal vertical vestibular-ocular response that demonstrates the integrity of the third nerve nuclei and confirms that the eye movement disorder is supranuclear. Internuclear ophthalmoplegia (INO), which is an adduction paresis on conjugate horizontal gaze (usually with abduction nystagmus of the contralateral eye), has been described previously in an apparently milder form in a series of 4 patients with PSP.”
“In our case, we could not elicit any effective eyeball movements in his eyes. From the patient’s serial photographs, it is evident that his ophthalmoplegia had been accompanied by progressive eyeball deviation in the upward and outward directions.”
“We suggest that the earliest ocular findings in PSP may be supranuclear gaze palsy, but as the disease progresses, nuclear level gaze control may also be affected, and exodeviation of the eyeballs may be a clinical manifestation in advanced PSP patients.”