Multiple system atrophy is a fatal neurodegenerative disorder that is rapidly progressive.1 It is mostly adult onset ranging from 30-90 years of age (average age of 55 years) and mostly affects men than women. The cause of the disorder is unknown, but interplay of environmental and genetic factors has been proposed. The progressive loss of nerve cells along with glial cytoplasmic inclusions develops in various areas of the central nervous system. These contain alpha-synuclein proteins that are significant in the pathogenesis of multiple system atrophy.
Prognosis For Multiple System Atrophy
The prognosis of patients with multiple system atrophy is poor as the progression of the disease is very rapid within 5-10 years of onset. Currently, there is no cure for the disorder and the treatments available are only symptomatic, which does not reverse or halt the condition. The survival rate is also around 6-10 years from the onset of first symptoms. When the disorder affects at a later stage of life, the duration of survival is also shorter. Although, MSA-P and MSA-C are both associated with the same survival period, the progression of MSA-P phenotype is more debilitating. Therefore, the loss of striatonigral cells is directly proportional to the severity of the disease. 2
The disease is characterized by symptoms related to Parkinson’s, cerebellar signs, autonomic nervous system failure. Based on which symptoms predominate at the initial presentation, it is divided into two main types.
MSA-P With Parkinsonian Phenotype. In this, the patient presents mostly symptoms of tremor, bradykinesia, rigidity and postural instability along with symptoms of orthostatic hypotension, balance problems, urinary dysfunction, coordination problems, dysphagia and dysarthria.
MSA-C With Cerebellar Phenotype. In this, the predominant symptoms are of loss of coordination and balance with cerebellar ataxia. There is problem with gait, balance, dysarthria, and dysphagia, nystagmus along with orthostatic hypotension, urinary dysfunction, tremors, and rigidity.
The additional symptoms of multiple system atrophy involve sleep apnea, snoring, inhalation sighs, forward flexion of neck (antecollis), lateral curvature of spine (Pisa syndrome), heat intolerance, reduced saliva, sweat or tears, fecal incontinence, hyperreflexia, spasticity, dystonia, REM sleep behavior disorder, and stridor. In severe cases, aspiration pneumonia, respiratory failure, deep vein thrombosis, pulmonary embolism and sudden death occur.
Generally, MSA-P is more common in most parts of the world; however, MSA-C is more common in Japan.
Treatment For Multiple System Atrophy
There is no cure for multiple system atrophy.3 The treatment is aimed at managing the symptoms of the disease. Parkinsonism is managed by levodopa and other drugs, such as amantadine, ropinirole, pramipexole. However, these drugs are more effective for classic Parkinson’s and even if they are effective for multiple system atrophy patients, the effectiveness diminishes over time.
Orthostatic hypotension is managed with dietary intake of salt, 16 ounces (approximately 500 ml) water consumption after waking up in the morning or after exertion, small, frequent meals, and head elevation while sleeping. If the above non-pharmacological methods do not work then medications such as fludrocortisone, midodrine, and ephedrine can be used to manage orthostatic hypotension.
- Urinary incontinence can be managed with oxybutynin or catheterization. Noctural polyuria is managed with desmopressin.
- Erectile dysfunction can be managed with sildenafil, tadafil or vardenafil
- Constipation can be managed with dietary fibers, laxatives, and lactulose.
- Dystonia can be managed with botulinum toxin injections
- Sleep apnea is managed with CPAP and REM sleep behavior disorder is managed with clonazepam
- Stridor and speech problems can be managed by speech therapy, occupational therapy or physiotherapy.
- Tracheostomy can be considered for respiratory stridor.
- Severe dysphagia with increased chances of aspiration can be managed with gastrostomy or cricopharyngeal myotomy.
Gait instructions should be given as the chances of fall increases with multiple system atrophy progression. Physical therapy and water based exercises are needed for deconditioning and performing daily activities. Wheelchair might be required for individuals unable to walk unassisted.
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