PD, such as early onset of dementia, early and severe balance problems, or inability to perform certain eye movements. Progressive supranuclear palsy : Is the most common of the Parkinson-plus syndromes. This condition results from degeneration of neurons in the basal ganglia, brain stem, thalamus, and frontal cortex. The underlying cause of this degeneration is unknown. Symptoms include postural instability leading to falls within the first year of disease onset and supranuclear ophthalmoplegia, a weakening of the vertical gaze, especially in the downward direction. Individuals with progressive supranuclear palsy have a median survival rate of 7 to 9 years from onset of symptoms (Fabbrini et al., 2019). Multiple system atrophy : Is a progressive, idiopathic, degenerative process, beginning in adulthood and presenting with varying degrees of parkinsonism, autonomic failure, cerebellar dysfunction, and pyramidal signs. It is poorly responsive to levodopa or dopamine agonists and has a median survival of 6.2 years, with a range of 0.5 to 24 years. It is characterized by symptoms of autonomic nervous system failure such as fainting spells and bladder control problems, along with cerebellar symptoms and early speech problems (Fabbrini et al., 2019). Striatonigral degeneration : Is a progressive degenerative disorder that is one type of multiple system atrophy. The major finding is parkinsonism with autonomic and cerebellar signs. It is not responsive to levodopa. Olivopontocerebellar atrophy : Involves the degeneration of the cerebellum, pons, and inferior olivary nucleus, resulting in ataxia, balance difficulty, abnormal eye movements, bowel or bladder problems, lightheadedness when standing, rigidity, and tremor. It is present in multiple system atrophy and prion
disorders (rare neurodegenerative diseases such as Creutzfeldt- Jakob Disease). Corticobasal ganglionic degeneration : Involves cortical atrophy of the frontal and parietal lobes and loss of dopamine in the substantia nigra. Typical symptoms are bradykinesia, tremor, focal rigidity, and marked dystonia, usually in one arm; limb apraxia is also common. The prognosis is for severe disability and death within 10 years (Fabbrini et al., 2019). Lewy body dementia : Involves the typical signs and symptoms of parkinsonism (bradykinesia, tremor, rigidity, and postural instability) and early onset of dementia. It is very difficult to differentially diagnose LBD, and individuals with this disease are usually first diagnosed with PD or AD. Hallucinations are more common in LBD, and their manifestation may lead the clinician to a differential diagnosis. As a result of the hallucinations, these individuals are sometimes hospitalized and given antipsychotic medication. Individuals with LBD have extreme sensitivity to antipsychotics, and this heightened sensitivity may be the clue that leads to a differential diagnosis (Walker et al., 2015). Individuals with LBD also tend to have earlier onset of more significant sleep disturbances (Scharre et al., 2016). A study comparing and contrasting motor and cognitive features of AD, PD, and LBD found that AD had more amnesia and orientation impairments but less executive and visuospatial deficits than LBD subjects. The LBD group had more sleepiness, cognitive/ behavioral fluctuations, hallucinations, and sleep apnea than the AD or PD groups. Axial motor, gait, and balance disturbances correlated with executive, visuospatial, and global cognition deficits and the Tinetti Mobility Test (TMT), Berg Balance Scale (BBS), and 9-Hole Peg Tests differentiated LBD from both AD and PD (Fritz et al., 2016; Scharre et al., 2016). Table 1 differentiates PD from other disorders of the basal ganglia based on commonly observed symptoms.
Table 1: Parkinson-Like Syndromes and Their Symptoms
Identified disorder*
Symptom
PD Yes Yes Yes Yes No
PSP Yes Yes Yes
MSA
SND
OPCA CBGD
LBD
Bradykinesia
Yes Yes Yes
Yes Yes Yes No No
Yes/no
Yes Yes Yes
Yes Yes Yes Yes No Yes
Rigidity
Yes Yes
Gait disturbance
Tremor Ataxia
No No
Yes/no
Yes/no
Yes/no
Yes Yes
Yes
No No
Dysautonomia
Yes/no Yes/no Yes/no Yes/no
Yes/no
Yes/no
Yes/no
Dementia
Yes Yes
Yes/no
No Yes
No Yes No No No
Yes/no
Yes/early
Dysarthria or dysphagia
Yes Yes No No
Yes Yes Yes Yes No Yes No No No Yes No No
Yes
Dystonia
Yes/no
Yes/no
Yes/no
Limb apraxia Myoclonus Neuropathy
No
No No No Yes
No No No No
No Yes No No Yes Yes Yes Yes Yes
Yes/no
No No
Yes/no
Yes/no
Oculomotility disturbance Orthostatic hypotension
Yes Yes Yes No
Yes
Yes/no Yes/no
Yes/no Yes/no
Yes/no Yes/no
Yes/no Yes/no
Sleep abnormality
Hallucinations
No**
No No
No No
No No No
Asymmetrical findings Levodopa response
Yes Yes No
Yes/no Yes/no Yes/no
Yes/no
Yes/no
Neuropathy No *PD = Parkinson’s disease; PSP = progressive supranuclear palsy; MSA = multiple system atrophy; SND = striatonigral degeneration; OPCA = olivo-pontocerebellar atrophy; CBGD = corticobasal ganglionic degeneration; LBD = Lewy body dementia ** Hallucinations are sometimes seen in PD, but are most often due to dopamine medications; decreased dosage usually resolves the hallucinations. Note . From Western Schools, 2018. No No Yes/no
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