On MRI, a hyperintense rim at the lateral edge of the dorsolateral putamen was seen in Atrophy of the corpus callosum was seen in a subpopulation of MSA, suggesting hemispheric involvement in a subgroup of MSA patients. The present study suggested that many factors are involved in the progression of MSA but, most importantly, the interval from initial symptom to combined motor and autonomic dysfunction can predict functional deterioration and survival in MSA.
Brundin et al, This hypothesis has been gaining considerable ground in recent years. It seems likely that tau spontaneously mutates into a pathologic form that recruits other tau and causes disease.
In other words, rather than being transmitted through "mad cow" nervous tissue, these disorders may, be spontaneous mutations that take 20 years to spread within one's nervous system. Depletion of catecholamine neurons in the rostral ventrolateral medulla is a consistant finding in patients with MSA and autonomic failure. Bennaroch et al, A consensus conference on the diagnosis of multiple system atrophy set out diagnostic criteria Gilman et al, ; There are three levels of certainty: Possible, probable and definate.
Rather, we think the main goal should be to identify people in whom MSA is worth considering, and following them to see if they are progressing. The diagnosis of definite MSA requires pathological confirmation i. This definition means that there are essentially no persons living with "definate" MSA. So far, there are no biomarkers for MSA. Potential ones include alpha-synuclein imaging and MRI morphometry.
The so-called "hot cross bun sign" is said to be specific for but not pathognomonic for MSA Burk et al, Figure 2 shows the sign in one of our patients felt to have MSA. A hot cross bun sign is also seen in some of the spinocerebellar atrophies. So in other words, if your sense of smell is normal, this is a point for MSA.
These observations need confirmation as they are based on too few patients. The most difficult differential diagnosis is between Parkinsons disease and levodopa-responsive striato-niagral degeneration-type MSA.
PSP is generally confirmed by eye-movement abnormalities. In early stages, Cortico-basal-ganglionic degeneration may be impossible to distinguish from MSA but as more cortical signs develop in later stages, the disorders may be possible to separate. We have encountered a patient with what we thought was CBGD, that turned out to have Jacob Creutzfeldt a prion disease. The rapid progression in this patient was probably the key.
Kim et al proided approximately 30 different conditions that might be confused with MSA -- basically all of neurology. The most common mimickers are:. According to Goldstein and others , orthostatic hypotension that occurs in Parkinsonism is due to cardiac sympathetic denervation, while in MSA, it caused by central regulatory disturbances. This is certainly not a common mimicker.
MSA patients either do not respond or respond poorly to levodopa. Doses of 1 to 1. Autonomic failure can be treated with salt supplements, florinef, and mitodrine. Mitodrine might work in the orthostatic hypotension of MSA as opposed to that of Parkinsonism, as in MSA the post-ganglionic sympathetics are retained Goldstein et al, It has been suggested that MSA patients may benefit from replacement of central norepinephrine or epinephrine with precursors such as dihidroxyphenylserine Benarroch et al, Many patients with MSA develop sleep-disordered breathing with stridor, due to bilateral vocal fold paresis.
For this reason, patients with MSA should get a sleep study. Treatment of the sleep disorder may include continuous positive airway pressure CPAP or tracheostomy. Multiple-System Atrophy Research, Most individuals with MSA and their caregivers attempt to make realistic plans anticipating a slow neurological decline.
Patients and caregivers should establish early on their wishes regarding invasive supportive care -- intubation, feeding tubes -- as these issues are almost certain to come up in the course of the disease.
Philadelphia, PA: Elsevier; chap Disorders of the autonomic nervous system. Brocklehurst's Textbook of Geriatric Medicine and Gerontology. URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. Learn more about A. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions.
Call for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright A. Any duplication or distribution of the information contained herein is strictly prohibited. Access myPennMedicine For Patients and Visitors. Patient Information. Conditions Treated A-Z. Multiple System Atrophy. Definition Multiple system atrophy- parkinsonian type MSA-P is a rare condition that causes symptoms similar to Parkinson disease.
MSA-P is most often diagnosed in men older than Symptoms MSA damages the nervous system. Your blood pressure will be taken while you are lying down and standing up.
0コメント