PORTO, PORTUGAL — Vascular parkinsonism displays a range of noncognitive symptoms, which explains why its diagnosis can depend on the bias of the specialist doing the evaluation, said Joseph Ghika, M.D., at the Fourth International Congress on Vascular Dementia.
The same group of symptoms might be referred to as vascular parkinsonism (or gait disorder) by movement disorder specialists, central incontinence by urologists, vascular depression by psychiatrists, apraxia of gait by neuropsychologists, gait disorder of hydrocephalus by neurosurgeons, cardiogenic dementia by cardiologists, senile gait disorder by geriatricians, and small- and/or large-vessel disease (or poststroke/multistroke dementia) by stroke specialists, said Dr. Ghika of the Centre Hospitalier Universitaire Vaudois in Lausanne, Switzerland.
Vascular parkinsonism accounts for 3%–6% of all Parkinson's disease (PD) cases. The evolution of vascular parkinsonism is more rapid than that of PD and may have a stepwise progression. Generally, patients with vascular parkinsonism are older than those with PD and have vascular risk factors. They are usually nonresponsive to dopa treatment.
Presentation may involve a number of symptoms that are not seen in other forms of cognitive impairment/dementia: gait disturbances (gait ignition failure, frontal gait disorder, frontal or subcortical disequilibrium), focal deficits, loss of sphincter control, emotional lability (forced laughter, pseudobulbar syndrome), and psychomotor slowing.
“It's a symmetrical axial/proximal Parkinson's that involves mostly the lower extremities,” said Dr. Ghika. Patients tend to be nontremulous except in posture and can have a mixture of rigidity and spasticity, with axial and proximal predominance without cogwheeling.
Associated gait disorders develop early in the course of degeneration, often at the same time that impairment of executive function becomes apparent. Patients have problems standing, starting to walk, and changing directions. Their steps are short and shuffling. Patients spread their feet in a wide base for standing and walking and turn without turning the trunk. Posture is stooped but without flexion at the hip or knee, unlike in PD. Patients have great difficulty getting up from a sitting position and are often unable to do so unassisted. There is marked retropulsion with a loss of protective/postural reflexes. Arm swing can be variable to increased. The hodgepodge of movement traits have hampered ongoing efforts to identify a characteristic pattern of gait disturbances for vascular parkinsonism. “It's a problem of ataxia and apraxia all together,” said Dr. Ghika.
Corticobulbar/pseudobulbar syndrome affects more than half of patients with vascular parkinsonism, taking the form of emotional lability and/or forced laughter. Their faces often carry a “mask” of bewilderment. Their speech pattern is typically low, slow, and monotonous. Their speech may be dysarthric, very nasal, aprosodic, and monosyllabic. Their verbal communication may be aspontaneous, or they may be mute, stutter, or have palilalia. Normal olfaction is absent.
Urinary dysfunction/incontinence is also common; half of patients with vascular parkinonism experience detrusor hyperreflexia. Dyskinesias are common. Hemichorea-hemiballism may be bilateral. Patients often have myoclonus upon startling. They have postural (action) or Holmes tremors.
Focal neurologic deficits are quite common, affecting about 34% of those with vascular dementia. As many as 63% of those with vascular parkinsonism have brisk reflexes, by some estimates. Other pyramidal signs include synkinesias, clonus, and spasticity. Hemiataxia and hemianopia may be present.
It can be difficult to identify dementia due to vascular parkinsonism. The differential diagnosis includes hydrocephalus, other dementias (Alzheimer's disease, frontotemporal dementia, etc.), atypical parkinsonism (progressive supranuclear palsy, corticobasal ganglionic degeneration), idiopathic dopa-responsive PD, multiple sclerosis/leukodystrophies, other white matter diseases, and motor neuron disease.