RUTH MAY DE VILLA BSPT - IV

PARKINSON-PLUS SYNDROME

1. Parkinson-Plus Syndrome – a group of neurodegenerative diseases can affect

the substantia nigra and produce parkinsonian symptoms along other
neurological signs. Some are the ff:

 Progressive supranuclear palsy (PSP)

o known as the Steele-Richardson-Olszewski syndrome.
o Olszewski, Steele, and Richardson reviewed autopsies of
patients who had a syndrome of pseudobulbar palsy, supranuclear
ocular palsy (chiefly affecting vertical gaze), extrapyramidal
rigidity, gait ataxia, and dementia. They found a consistent pattern
of neuronal degeneration and neurofibrillary tangles (NFTs),
chiefly affecting the pons and midbrain.

 Cortical-basal ganglionic degeneration

o It is characterized pathologically by enlarged achromatic
neurons in cortical areas (particularly parietal and frontal lobes)
along with nigral and striatal neuronal degeneration. The onset is
insidious and typically unilateral, with marked rigidity dystonia on
the involved arm. Cortical signs of apraxia, alien limb phenomena,
cortical sensory loss, cortical reflex myoclonus, and occasionally
aphasia are also seen. Speech is hesitant, gait is poor, and
occasionally action tremor is evident.

 Hemi parkinsonism-hemiatrophy syndrome

o This relatively benign syndrome consists of
hemiparkinsonism in association with ipsilateral body hemiatrophy
or contralateral brain hemiatrophy. The parkinsonism usually
begins in young adults and often remains as hemiparkinsonism,
sometimes with hemidystonia. It tends to be nonprogressive or
slowly progressive compared with PD. The disorder is thought to
result from brain injury early in life, possibly even perinatally. It
sometimes responds to medications.

 Normal Pressurem Hydrocephalus

o Features of urinary incontinence and dementia occur
later. Tremor is rare. The grossly enlarged ventricles lead to the
correct diagnosis, with the symptoms often improving on removal
or shunting of CSF. The gait disorder is in striking contrast to the
lack of parkinsonism in the upper part of the body. The major
differential diagnoses for lower body parkinsonism include
vascular parkinsonism and the idiopathic gait disorder of the
elderly.

 Disorders with cerebellar/autonomic/pyramidal manifestations:

 Also called Multiple System Atrophy (MSA)
  has been applied to a group of four syndromes previously
considered distinct and separate entities:

o striatonigral degeneration – pure parkinsonism,

parkinsonism without tremor.

o Shy-Drager syndrome – autonomic dysfunction,

preganglionic sympathetic neurons are lost, but the
postganglionic sympathetic neurons are intact.
Orthostatic hypotension is a major disabling symptom

o Olivopontocerebellar Atrophy (OPCA) – ataxia-, a mixture

of parkinsonism and cerebellar syndrome

o parkinsonism-amyotrophy syndrome – amyotrophy -

progressive wasting of muscle tissues. Muscle pain is
also a symptom

 Disorders with prominent and often early dementia:

 Diffuse Lewy body disease (DLBD) - a progressive

neurodegenerative disorder characterized by the presence of
parkinsonian symptoms and neuropsychiatric disturbances
commonly accompanied by dementia.

 Alzheimer’s disease - the most common cause of dementia, is

an acquired cognitive and behavioral impairment of sufficient
severity that markedly interferes with social and occupational
functioning.