[MIM 146 500]

(Shy-Dräger syndrome, Parkinson Plus disease)

Prevalence: 3-5 cases/100,000 in Europe and in USA, 13/100,000 in Japan. Unknown cause. Onset between 30 and 50 years of age. Neurologic disease caused by progressive loss of neurons in different parts of the brain, hence the name of the disease. There is also  accumulation of α-synuclein in the oligodendrocytes.

According to the initial neurologic anomalies associated with the dysautonomic disorders, there are 2 phenotypes:

-        P or I: Parkinson type (80 %), also called Parkinson Plus syndrome: parkinsonian signs responding poorly if not at all to levodopa

-        C or II: cerebellar type (20 %): disorders of stance and gait, dysarthria

Dysautonomic disorders are:

-        erectile and urinary disorders, gastroparesis

-        orthostatic hypotension with sometimes hypertension when supine

-        disorders of swallowing, paralysis or hypertonia of the vocal cords

-        sleep disorders: agitation, words or laryngeal stridor during REM phase sleep, obstructive or central apnea, Cheyne-Stokes breathing

-        reduction of the production of sweat, tears and saliva

-        constipation or diarrhea

Treatments:

-        levodopa for 'P' forms

-        orthostatic hypotension: midodrine (α1-agonist, last intake 5 h before bedtime), fludrocortisone (50 to 150 µg/d), pyridostigmine (30 mg 2-3 x/day), droxidopa

-        urinary disorders: oxybutinine

Reference center: http://www.chu-toulouse.fr/-centre-de-reference-de-l-atrophie-multisystematisee

Anesthetic implications:

all daily treatments must be continued until the day of surgery; risk of central and obstructive apnea; risk of aspiration; major risk of difficulties to control hypo or hypertension; eye protection; sometimes heart rate responding poorly to atropine; risk of perioperative hypo- and hyperthermia.

References :

-        Lim YS, Kennedy NJ.
Multiple system atrophy as a cause of upper airway obstruction.
Anaesthesia 2007; 62: 1179-82

-        Gomesz FA, Montell M.
Caudal anesthesia in the Shy-Drager syndrome.
Anaesthesia 1992; 47: 1100

-        Niquille M, Van Gessel E, Gamulin Z.
Continuous spinal anesthesia for hip surgery in a patient with Shy-Drager syndrome.
Anesth Analg 1998; 87: 396-9.

-        Kumar CM, Seet E, Kumar Singh R, Joshi GP.
Anesthesia in patients with multiple-system atrophy: a narrative review and practice guidance.
A&A Practice, in press

Updated: December 2018