Parkinson plus syndrome
Encyclopedia
Parkinson-plus syndromes, also known as disorders of multiple system degeneration, are a group of neurodegenerative diseases featuring the classical features of Parkinson's disease
(tremor
, rigidity
, akinesia/bradykinesia, postural instability) with additional features that distinguish them from simple idiopathic
Parkinson's disease. Some consider Alzheimer's disease
to be in this group.
The atypical parkinsonian or Parkinson Plus syndromes are often difficult to differentiate from Parkinson's disease and each other. They include multiple system atrophy
(MSA), progressive supranuclear palsy
(PSP), and corticobasal degeneration
(CBGD). Dementia with Lewy bodies
(DLB), may or may not be part of the PD spectrum, but it is increasingly recognized as the second most common type of neurodegenerative dementia after Alzheimer's disease. These disorders are currently lumped into two groups, the synucleinopathies and the tauopathies.
Additional Parkinson-plus syndromes include Pick's disease
and olivopontocerebellar atrophy
(OPCA). The latter is characterized by ataxia
and dysarthria
, and may occur either as an inherited disorder or as a variant of multiple system atrophy. MSA is also characterized by autonomic
failure, formerly known as Shy-Drager syndrome.
Nuclear medicine
SPECT procedure using 123I
-IBZM
, is an effective tool in the establishment of the differential diagnosis between patients with Parkinson's disease and Parkinson-Plus syndromes.
s may respond to medication
s not used in Parkinson's disease.
Parkinson's disease
Parkinson's disease is a degenerative disorder of the central nervous system...
(tremor
Tremor
A tremor is an involuntary, somewhat rhythmic, muscle contraction and relaxation involving to-and-fro movements of one or more body parts. It is the most common of all involuntary movements and can affect the hands, arms, eyes, face, head, vocal folds, trunk, and legs. Most tremors occur in the...
, rigidity
Rigidity
Rigid or rigidity may refer to:*Stiffness, the property of a solid body to resist deformation, which is sometimes referred to as rigidity*Structural rigidity, a mathematical theory of the stiffness of ensembles of rigid objects connected by hinges...
, akinesia/bradykinesia, postural instability) with additional features that distinguish them from simple idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
Parkinson's disease. Some consider Alzheimer's disease
Alzheimer's disease
Alzheimer's disease also known in medical literature as Alzheimer disease is the most common form of dementia. There is no cure for the disease, which worsens as it progresses, and eventually leads to death...
to be in this group.
The atypical parkinsonian or Parkinson Plus syndromes are often difficult to differentiate from Parkinson's disease and each other. They include multiple system atrophy
Multiple system atrophy
Multiple system atrophy is a degenerative neurological disorder. MSA is associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and other autonomic functions of the body such as bladder control or blood pressure...
(MSA), progressive supranuclear palsy
Progressive supranuclear palsy
Progressive supranuclear palsy is a degenerative disease involving the gradual deterioration and death of specific areas of the brain....
(PSP), and corticobasal degeneration
Corticobasal degeneration
Corticobasal degeneration or Corticobasal Ganglionic Degeneration is a rare progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia. It is characterized by marked disorders in movement and cognitive dysfunction...
(CBGD). Dementia with Lewy bodies
Dementia with Lewy bodies
Dementia with Lewy bodies , also known under a variety of other names including Lewy body dementia, diffuse Lewy body disease, cortical Lewy body disease, and senile dementia of Lewy type, is a type of dementia closely allied to both Alzheimers and Parkinson's Diseases...
(DLB), may or may not be part of the PD spectrum, but it is increasingly recognized as the second most common type of neurodegenerative dementia after Alzheimer's disease. These disorders are currently lumped into two groups, the synucleinopathies and the tauopathies.
Additional Parkinson-plus syndromes include Pick's disease
Pick's disease
Pick's disease, is a rare neurodegenerative disease that causes progressive destruction of nerve cells in the brain. Symptoms include loss of speech , and dementia. While some of the symptoms can initially be alleviated, the disease progresses and patients often die within two to ten years...
and olivopontocerebellar atrophy
Olivopontocerebellar atrophy
Olivopontocerebellar atrophy is a term used to define neuronal degeneration in the cerebellum, pontine nuclei, and inferior olivary nucleus...
(OPCA). The latter is characterized by ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
and dysarthria
Dysarthria
Dysarthria is a motor speech disorder resulting from neurological injury of the motor component of the motor-speech system and is characterized by poor articulation of phonemes...
, and may occur either as an inherited disorder or as a variant of multiple system atrophy. MSA is also characterized by autonomic
Autonomic nervous system
The autonomic nervous system is the part of the peripheral nervous system that acts as a control system functioning largely below the level of consciousness, and controls visceral functions. The ANS affects heart rate, digestion, respiration rate, salivation, perspiration, diameter of the pupils,...
failure, formerly known as Shy-Drager syndrome.
Nuclear medicine
Nuclear medicine
In nuclear medicine procedures, elemental radionuclides are combined with other elements to form chemical compounds, or else combined with existing pharmaceutical compounds, to form radiopharmaceuticals. These radiopharmaceuticals, once administered to the patient, can localize to specific organs...
SPECT procedure using 123I
Iodine-123
Iodine-123 is a radioactive isotope of iodine used in nuclear medicine imaging, including single photon emission computed tomography . The isotope's half-life is 13.22 hours; the decay by electron capture to tellurium-123 emits gamma radiation with predominant energies of 159 keV and 127 keV...
-IBZM
IBZM
IBZM is a chemical substance. Pharmaceutically it is a dopamine antagonist and it can be used by nuclear medicine physicians as a radioactive tracer for SPECT where the radioactive isotope is iodine-123 or iodine-125...
, is an effective tool in the establishment of the differential diagnosis between patients with Parkinson's disease and Parkinson-Plus syndromes.
Treatment
The Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to anti-parkinsonian medication than Parkinson's disease. However, the additional features of the diseaseDisease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...
s may respond to medication
Medication
A pharmaceutical drug, also referred to as medicine, medication or medicament, can be loosely defined as any chemical substance intended for use in the medical diagnosis, cure, treatment, or prevention of disease.- Classification :...
s not used in Parkinson's disease.