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Shy-Drager SyndromeVentilators, MechanicalSyndromeShynessMultiple System AtrophyPhysical and Rehabilitation MedicineEncyclopedias as TopicSpeech TherapyOccupational TherapySpeech DisordersManebManganese PoisoningZinebManganeseFacilitated DiffusionDyskinesias
AutonomicClinicallyNeurodegenerative disordersClinicalParkinsonParkinson'sOlivopontocerebellarOnsetPrognosisTremorInclusionProgressiveNeurologicCommonTreatmentMultipleCardiacManagementMadeBasal gangliaAutonomic failureMultisystemParkinson PlusParkinsonismIdiopathicSupranuclearFeaturesPMIDSpinalGeneticTreatment
Autonomic4
  • The presence of inclusion bodies known as Papp-Lantos bodies, in the movement, balance, and autonomic-control centres of the brain are the defining histopathologic hallmark of MSA. (wikipedia.org)
  • MSA is also characterized by autonomic failure, formerly known as Shy-Drager syndrome. (wikipedia.org)
  • Other manifestations of multiple system atrophy are Shy-Drager syndrome, in which autonomic failure predominates, and sporadic olivopontocerebellar degeneration, which is characterized primarily by cerebellar signs. (medscape.com)
  • The group of disorders classified under multiple system atrophy belong to a broader class called the Parkinson-plus syndromes , which have in common features such as bradykinesia and rigidity seen in Parkinson disease, combined with additional components such as autonomic dysfunction, ataxia, or dementia. (medscape.com)
Clinically1
  • Diagnosis is made clinically, based on history and neurologic exam. (bmj.com)
Neurodegenerative disorders1
  • Proper diagnosis of these neurodegenerative disorders is important as individual treatments vary depending on the condition. (wikipedia.org)
Clinical4
  • Clinical features that distinguish Parkinson-plus syndromes from idiopathic PD include symmetrical onset, a lack of or irregular resting tremor, and a reduced response to dopaminergic drugs (including levodopa). (wikipedia.org)
  • The consensus group described the clinical features of the disease and set the criteria for diagnosis of possible, probable, and definite multiple system atrophy. (medscape.com)
  • Parkinsonism is a clinical syndrome characterized by tremor , bradykinesia (slowed movements), rigidity , and postural instability . (wikipedia.org)
  • Originally considered as primarily a pediatric disease, neuronal intranuclear hyaline inclusion disease has been increasingly recognized to be a heterogeneous disease with highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. (radiopaedia.org)
Parkinson15
  • Parkinson-plus syndromes (PPS) are a group of neurodegenerative diseases featuring the classical features of Parkinson's disease (tremor, rigidity, akinesia/bradykinesia, and postural instability) with additional features that distinguish them from simple idiopathic Parkinson's disease (PD). (wikipedia.org)
  • Parkinson-plus syndromes are either inherited genetically or occur sporadically. (wikipedia.org)
  • Atypical parkinsonism and other Parkinson-plus syndromes are often difficult to differentiate from PD and each other. (wikipedia.org)
  • Additional Parkinson-plus syndromes include Pick's disease and olivopontocerebellar atrophy. (wikipedia.org)
  • Accurate diagnosis of these Parkinson-plus syndromes is improved when precise diagnostic criteria are used. (wikipedia.org)
  • Since diagnosis of individual Parkinson-plus syndromes is difficult, the prognosis is often poor. (wikipedia.org)
  • The nuclear medicine SPECT procedure using 123I‑iodobenzamide (IBZM), is an effective tool in the establishment of the differential diagnosis between patients with PD and Parkinson-plus syndromes. (wikipedia.org)
  • Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to antiparkinsonian medication than PD. (wikipedia.org)
  • citation needed] Current therapy for Parkinson-plus syndromes is centered around a multidisciplinary treatment of symptoms. (wikipedia.org)
  • Parkinson disease is by far the most common cause of the parkinsonian syndrome , accounting for approximately 80% of cases (the remainder being due to other neurodegenerative diseases, such as Lewy body dementia ) 1 . (radiopaedia.org)
  • However, because of the common parkinsonian features, the disorders have been collectively named Parkinson-plus syndromes. (medscape.com)
  • Parkinson-plus syndromes respond poorly to the standard treatments for Parkinson disease (PD). (medscape.com)
  • An inadequate response to treatment in a patient with parkinsonian symptoms suggests the possibility of a Parkinson-plus syndrome and warrants a search for the signs and symptoms of degeneration in other neuronal systems. (medscape.com)
  • Modern immunocytochemical techniques and genetic findings suggest that Parkinson-plus syndromes can be broadly grouped into 2 types: synucleinopathies and tauopathies. (medscape.com)
  • See the related images below regarding Parkinson-plus syndromes. (medscape.com)
Parkinson's2
  • The most common first sign of MSA is the appearance of an "akinetic-rigid syndrome" (i.e. slowness of initiation of movement resembling Parkinson's disease) found in 62% at first presentation. (wikipedia.org)
  • Parkinson's syndrome" redirects here. (wikipedia.org)
Olivopontocerebellar1
  • Thirty years later, the first consensus statement on the diagnosis of multiple system atrophy, by Gilman et al, recommended that the term striatonigral degeneration be replaced with multiple system atrophy with predominantly parkinsonian features (MSA-P) and that the term sporadic olivopontocerebellar degeneration be replaced with multiple system atrophy with predominantly cerebellar features (MSA-C). (medscape.com)
Onset2
  • Additional features include bradykinesia, early-onset postural instability, increased rigidity in axial muscles, dysautonomia, alien limb syndrome, supranuclear gaze palsy, apraxia, involvement of the cerebellum including the pyramidal cells, and in some instances significant cognitive impairment. (wikipedia.org)
  • Nevertheless, since the usefulness of skin biopsy and brain MRI for the diagnosis of NIID has been described, the number of NIID diagnoses has increased, in particular adult-onset NIID 4 . (radiopaedia.org)
Prognosis1
  • When an employee faces a serious health problem or is told he or she needs surgery, seeking a 2nd opinion from another physician can, in some cases, have a significant impact on their diagnosis, treatment plan or prognosis. (surgerysecondopinion.com)
Tremor1
  • Conventional brain MRI findings of patients with neuronal intranuclear hyaline inclusion disease strongly resemble those seen in fragile X-associated tremor/ataxia syndrome (FXTAS) , including symmetric white matter involvement in combination with hyperintense changes of the middle cerebellar peduncles 4 . (radiopaedia.org)
Inclusion1
  • [ 3 ] The identification of these inclusion bodies was a unifying factor in the pathologic classification of the 3 entities that now fall under the category of multiple system atrophy: MSA-P, MSA-C, and, less formally, Shy-Drager syndrome. (medscape.com)
Progressive1
  • This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. (wikipedia.org)
Neurologic1
  • In the evaluation of simple syncope and a normal neurologic evaluation, do not obtain brain imaging studies (computed tomography or magnetic resonance imaging). (aafp.org)
Common3
  • MSA is distinct from multisystem proteinopathy, a more common muscle-wasting syndrome. (wikipedia.org)
  • Medical 2nd opinion for medical or surgical diagnoses are exceedingly common, and even when they don't lead to another treatment plan, they're a worthwhile exercise. (surgerysecondopinion.com)
  • It's not common that treatment for most health problems, including most cancers, needs to start immediately after diagnosis. (surgerysecondopinion.com)
Treatment1
  • Not seeking a 2nd opinion in the case of a serious, complex, or rare diagnosis or recommendation for elective surgery is a missed opportunity to lower the risk of misdiagnosis and inappropriate or ineffective treatment. (surgerysecondopinion.com)
Multiple2
  • MSA is also different from multiple organ dysfunction syndrome, sometimes referred to as multiple organ failure, and from multiple organ system failures, an often-fatal complication of septic shock and other severe illnesses or injuries. (wikipedia.org)
  • In 2008, the second consensus statement on the diagnosis of multiple system atrophy was published. (medscape.com)
Cardiac1
  • Implantable loop recorders increase diagnostic yield, reduce time to diagnosis, and are cost-effective for suspected cardiac syncope and unexplained syncope. (aafp.org)
Management1
  • During their twice-weekly rounds, Dr Stern and other members of the Consultation Service discuss the diagnosis and management of conditions confronted. (psychiatrist.com)
Made1
  • The diagnosis can be made using peripheral nerve biopsy (e.g. sural nerve) 1 or myenteric plexus biopsy (e.g. rectal biopsy) 2,3 . (radiopaedia.org)
Basal ganglia1
  • This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. (wikipedia.org)
Autonomic failure2
Multisystem1
Parkinson Plus16
  • Parkinson-plus syndromes (PPS) are a group of neurodegenerative diseases featuring the classical features of Parkinson's disease (tremor, rigidity, akinesia/bradykinesia, and postural instability) with additional features that distinguish them from simple idiopathic Parkinson's disease (PD). (wikipedia.org)
  • Parkinson-plus syndromes are either inherited genetically or occur sporadically. (wikipedia.org)
  • Atypical parkinsonism and other Parkinson-plus syndromes are often difficult to differentiate from PD and each other. (wikipedia.org)
  • Additional Parkinson-plus syndromes include Pick's disease and olivopontocerebellar atrophy. (wikipedia.org)
  • Clinical features that distinguish Parkinson-plus syndromes from idiopathic PD include symmetrical onset, a lack of or irregular resting tremor, and a reduced response to dopaminergic drugs (including levodopa). (wikipedia.org)
  • Accurate diagnosis of these Parkinson-plus syndromes is improved when precise diagnostic criteria are used. (wikipedia.org)
  • Since diagnosis of individual Parkinson-plus syndromes is difficult, the prognosis is often poor. (wikipedia.org)
  • The nuclear medicine SPECT procedure using 123I‑iodobenzamide (IBZM), is an effective tool in the establishment of the differential diagnosis between patients with PD and Parkinson-plus syndromes. (wikipedia.org)
  • Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to antiparkinsonian medication than PD. (wikipedia.org)
  • citation needed] Current therapy for Parkinson-plus syndromes is centered around a multidisciplinary treatment of symptoms. (wikipedia.org)
  • 6] Perez et al report vocal tremor in 55% of patients with Parkinson disease and in 64% of patients with Parkinson-plus syndromes. (medscape.com)
  • However, because of the common parkinsonian features, the disorders have been collectively named Parkinson-plus syndromes. (medscape.com)
  • Parkinson-plus syndromes respond poorly to the standard treatments for PD. (medscape.com)
  • An inadequate response to treatment in a patient with parkinsonian symptoms suggests the possibility of Parkinson-plus syndrome and warrants a search for the signs and symptoms of degeneration in other neuronal systems. (medscape.com)
  • Modern immunocytochemical techniques and genetic findings suggest that Parkinson-plus syndromes can be broadly grouped into 2 types: synucleinopathies and tauopathies. (medscape.com)
  • See the related images below regarding Parkinson-plus syndromes. (medscape.com)
Parkinsonism2
  • Because the root cause and treatment for each form of parkinsonism is different, it's critical to obtain an accurate diagnosis. (davisphinneyfoundation.org)
  • As the name suggests, the cause of drug-induced parkinsonism (DIP) is due to the side effects of medications that affect the dopamine levels in the brain. (davisphinneyfoundation.org)
Idiopathic1
  • The most common initial diagnosis is idiopathic Parkinson disease. (medscape.com)
Supranuclear2
  • Progressive Supranuclear Palsy (PSP), also called Stelle-Richardson-Olszewski Syndrome, reflects the combined names of the scientists who defined the disorder in 1964. (davisphinneyfoundation.org)
  • Additional features include bradykinesia, early-onset postural instability, increased rigidity in axial muscles, dysautonomia, alien limb syndrome, supranuclear gaze palsy, apraxia, involvement of the cerebellum including the pyramidal cells, and in some instances significant cognitive impairment. (wikipedia.org)
Features1
  • The diagnosis of MSA is based mainly on clinical features. (medscape.com)
PMID3
  • PMID- 5097502 TI - A pericentric inversion, 5 p-q+, and additional complex rearrangements in a case of cri-du-chat syndrome. (nih.gov)
  • PMID- 5097514 TI - Differential representation of chromosomal HRNA in nuclear sap. (nih.gov)
  • PMID- 5097523 TI - Cell size distribution in brain suspensions and in fractions enriched with neuronal and glial cells. (nih.gov)
Spinal1
  • Neuropathological hallmarks of MSA are cell loss in the striatonigral and olivopontocerebellar structures of the brain and spinal cord accompanied by profuse, distinctive glia cytoplasmic inclusions (GCIs) formed by fibrillized alpha-synuclein proteins (defined as primary alpha-synucleinopathy). (medscape.com)
Genetic3
  • The diagnosis of SCA2 rests on the use of molecular genetic testing to detect an abnormal CAG trinucleotide repeat expansion in ATXN2 . (nih.gov)
  • Prenatal testing for a pregnancy at increased risk is possible if the diagnosis has been established by molecular genetic testing in an affected family member. (nih.gov)
  • thus, diagnosis depends on molecular genetic testing . (nih.gov)
Treatment1
  • Because physicians need to rule out many other conditions, it may take time to determine a correct diagnosis and the appropriate treatment. (davisphinneyfoundation.org)