What is the best course of management for an elderly patient with progressive neurologic decline, difficulty swallowing, and newly diagnosed vertical gaze palsy, suggestive of progressive supranuclear palsy (PSP)?

Management of Progressive Supranuclear Palsy with Vertical Gaze Palsy

For an elderly patient with progressive neurologic decline, dysphagia, and vertical gaze palsy consistent with progressive supranuclear palsy (PSP), initiate symptomatic treatment with levodopa while focusing on fall prevention, multidisciplinary supportive care for dysphagia, and early referral to palliative care given the poor prognosis and rapid disease progression. 1, 2, 3

Confirm the Diagnosis

Vertical gaze palsy, particularly affecting downward gaze more than upward gaze, is the hallmark diagnostic feature of PSP. 4, 1 The American Academy of Neurology emphasizes that this finding, combined with early postural instability and frequent falls, strongly suggests PSP rather than other parkinsonian syndromes. 1

Key Diagnostic Features to Document:

• Vertical supranuclear gaze palsy (downward > upward), with absent or slowed downward saccades and absent optokinetic nystagmus 4, 1
• Early postural instability with backward falls (not forward falls, which distinguishes PSP from other conditions) 5, 2
• Parkinsonism with bradykinesia, axial rigidity (especially neck stiffness), and gait disturbance 1, 6
• Pseudobulbar palsy manifesting as dysphagia and dysarthria 6, 3
• Frontal release signs including Myerson's sign, cognitive/behavioral changes, and executive dysfunction 2, 3

Obtain Brain MRI:

Order MRI of the brain to look for the "hummingbird sign" (midbrain atrophy with preserved pons) on sagittal images, which supports the diagnosis of PSP. 2 A midbrain area less than 70 mm² is consistent with PSP. 2

Initiate Pharmacologic Treatment

Start levodopa therapy despite the characteristically poor response in PSP. 1, 2, 3 While PSP typically shows minimal improvement with levodopa compared to Parkinson's disease, a trial is warranted as some patients may experience modest benefit in motor symptoms. 3

• Begin with carbidopa-levodopa 25/100 mg three times daily, titrating as tolerated 2
• Set realistic expectations: most PSP patients show limited or no response to levodopa 1, 3
• Monitor for any improvement in bradykinesia or rigidity over 3-4 months 3

Address Fall Risk Immediately

Falls are the most dangerous and common complication in PSP, occurring early in the disease course and often resulting in severe trauma. 5, 2, 6

Implement Fall Prevention Strategies:

• Physical therapy evaluation for gait training and assistive device prescription (walker with wheels and seat) 2, 3
• Home safety assessment to remove fall hazards and install grab bars 2
• Consider weighted walker to address the characteristic backward falls 5, 2
• Educate caregivers that PSP patients have decreased perception of danger due to frontal lobe dysfunction, leading to more severe fall-related injuries 2

The median time from symptom onset to first assessment is typically 3 years, but falls begin much earlier. 6 Early recognition and intervention are critical to prevent traumatic injuries like the hemopneumothorax described in recent case reports. 2

Manage Dysphagia Aggressively

Dysphagia is present in 80% of PSP patients and represents a major cause of morbidity and mortality. 6

• Speech-language pathology referral for swallowing evaluation and modified barium swallow study 3
• Diet modification with thickened liquids and pureed foods as needed 3
• Discuss aspiration risk and pneumonia prevention strategies 6, 3
• Early discussion of feeding tube placement should occur given the progressive nature of dysphagia in PSP 3

Address Ocular Symptoms

While the vertical gaze palsy itself typically does not require surgical intervention, associated ocular symptoms warrant attention:

• Eyelid abnormalities including blepharospasm, apraxia of eyelid opening/closing, and lid retraction are common 7, 3
• Botulinum toxin injections can be considered for symptomatic blepharospasm 7
• Artificial tears for exposure keratopathy from lid retraction 7

The ophthalmologic guidelines emphasize that supranuclear gaze palsies like PSP require neurological workup rather than strabismus surgery. 8

Cognitive and Behavioral Management

Executive dysfunction and frontal lobe impairment dominate the neuropsychological profile of PSP. 3

• Neuropsychological testing to establish baseline cognitive function 3
• Apathy and impulsivity are common and significantly impact caregivers 3
• Verbal fluency deficits are the most distinct cognitive impairment 3
• Consider psychiatric consultation for behavioral management 3

Prognosis and Palliative Care

PSP has a poor prognosis with median disease duration of 6-10 years depending on subtype. 9 The most aggressive subtype (PSP-PF, with early postural and frontal dysfunction) has a median survival of only 6 years. 9

Early Palliative Care Referral:

• Discuss prognosis openly with patient and family 6, 3
• Address goals of care early in the disease course 3
• Sleep disturbances and hallucinations are associated with increased mortality risk 6
• Quality of life focus given the progressive, incurable nature of the disease 2, 3

Critical Pitfall to Avoid

The most important clinical pitfall is delaying diagnosis in elderly patients with frequent falls. 2 PSP typically requires 3-4 years for diagnosis, yet falls appear much earlier than in other degenerative parkinsonian disorders. 2 A thorough neurological examination and sagittal brain MRI should be performed in any elderly patient with repeated unexplained falls to consider PSP, as early diagnosis may prevent severe trauma and improve quality of life. 2