A 71 y/o woman with downward gaze palsy, dementia, and recurrent falls
Kam Newman, Hesam Tabrizi, Parham Naghedechi, Kia Javaheri, Saeed Kahkeshani
Learning Objectives
1. Understanding the differences between
progressive supranuclear paly (PSP) and
parkinson disease (PD).
2- Recognizing the movement disorder
patterns in PSP.
Summary
Olszewski, Steele, and Richardson reviewed
autopsied of patients who had a syndrome of
pseudobulbar palsy, supranuclear ocular
palsy, extrapyramidal rigidity, gait ataxia,
and dementia.They found a consistent pattern
of neuronal degeneration and neurofiblliray
tangles, chiefly affecting the pons and
midbrain. It is clear that PSP continues to be
an under-recognized disorder with multilevel
involvement of the neuraxis that helps
differentiate it from other akinetic-rigid
syndromes such as PD. After PD, PSP is the
most common form of Parkinsonism with a
prevalence of 10% of that of PD. It is an
increasingly recognized akinetic-rigid
syndrome with features that may initially
mimic those of idiopathic PD. The clinical
hallmarks of PSP are vertical gaze palsy,
psudobulbar palsy, axial rigidity, and
cognitive impairment.
Acknowledgements
The authors wish to thank Dr. Hamid Iranmanesh, and Dr. Kaveh
Bahmanpour, Department of Family Medicine for their support.
San Jacinto Methodist Hospital, Baytown, TX
Case History
The patient is a 71 year old Caucasian female
with past history of walking problem and
dementia who was admitted hospital for altered
mental status. Her problems started with
recurrent falls and the ability to walk gradually
declined to the point that she stopped walking
completely about 5 to 6 month ago and she had to
use wheel chair. She has been declining in her
mental function since a couple of years ago, and
was treating for Alzheimer disease for about a
year. According to her family, she has also been
using diapers for several months. Also they have
noticed that during the past several month, her
arms became stiff and fingers flexed so that she
could not open her fist.
On physical exam, patient stares at the examiner
all the time and is not communicative. She
recognizes some family members around her, but
completely disoriented to the place and time, and
follows just some simple commands like
squeezing fingers. Her speech is slow and
somewhat slurred. She has a very poor eye
movement in all directions. In motor exam, she is
rigid in a generalized fashion, and her rigidity
was seen in all extremities and trunk. She holds
her right hand in a completely flexed position.
Deep tendon reflexes are exaggerated in all four
extremities. CBC, CMP, cardiac profile, LFT,
SPEP, RPR, CEA, and spinal tap all were
unremarkable. MRI of the brain revealed
atrophy of the midbrain.
Reference:
2. Clinical aspects of progressive supranuclear
palsy. Handb of Clin Neurol. 2008; 89:461-73.
Images
Oculocephalic maneuver demonstrates intact
reflex downgaze in a patient unable to look down
voluntarily.
New radiologic sign for the diagnosis of PSP.
Midsagittal MR image of a patient with PD (A) does
not show any apparent abnormality, while that’s of a
patient with PSP (B) shows marked atrophy of
midbrain tegmentum, and a patient with multiple-
system atrophy of the Parkinson type (MSA-P) shows
marked atrophy of pons (C). The midbrain to pons
ratio is always small in the patients with PSP. In pts
with PSP, the shapes of midbrain tegmentum (bird’s
head) and pons (bird’s body) on midsagittal MR
images look like a lateral view of a standing penguin
(especially the king penguin) with a small head and
big body.
Recognition of this penguin silhouette sign should
strongly raise suspicion for the diagnosis of PSP.
Discussion
Patients with PSP have an akinetic rigid Parkinson-
like syndrome, rest tremor is uncommon. The
symptoms consist of bradykinasia, decreased fine
motor skills, and hypophonia. Gait difficulties
progress faster than in PD. Unlike typical PD, falls
begin within the first year. Presentation can range
from unexpected falls, to visual (blurry or double
vision), cognitive (apathy, dementia), or
Parkinsonian symptoms with little or no tremor.
Compared with PD, PSP patients have more axial
than appendicular rigidity, more dystonia and a
more symmetric exam. Weak voice with
psudobulbar features can develop early;
swallowing problem develop later. Downgaze is
affected before upgaze, whereas lateral movements
of eye are usually preserved. Motor examination
reveals axial hypertonia that is out of proportion to
limb tone, which may be normal in contrast to PD
Patients. Eyelid opening and closing aparaxias are
far more common in PSP than in any other
extrapyramidal disorder. “Applause sign” helps to
discriminate PSP from FTD and PD. Pseudobulbar
palsy is an important symptom of PSP associateed
with high morbidity and decreased survival.
The pathology of PSP is characterized by
widespread neurodegeneration associated with tau
protein deposition in subcortical regions that
include the SN, globus pallidus, subthalamic
nucleus, midbrain, pontine reticular formation,
dentate nucleus of the cerebellum and the superior
cerebellar peduncle.
Compared with PD, PSP pts drive a limited benefit
from dopaminomimetics; nonethless in individual
patients this benefit can be clinically important.
Beyond dopamine, GABA receptors seem to be
decreased in the frontal cortex of PSP patients.