Too hot to handle: heat stroke in multiple system

Sirs: Autonomic dysfunction is acommon feature of multiple sys-tem atrophy (MSA). It can beincapacitating for patients, leadingto urinary urge, severe constipa-tion, altered sweating, and ortho-static hypotension with falls due tosyncope [1]. Here, we present anunusual complication of auto-nomic dysfunction in a patientwith definite MSA that came tolight during the unusual hotsummer of 2003 in the Nether-lands.A sixty-six year old man with afour year history of bladderhypocontractility, and a one yearhistory of orthostatic hypotension,was referred to our Department ofNeurology for further evaluation.The patient’s main subjectivecomplaint was lightheadednesswhile walking the stairs. He didnot report muscle stiffness orslowness when walking. Hisupright posture had becomeslightly stooped during thepreceding year. He furthercomplained of a dry mouth, beingunable to sweat, impotence, andproblems with swallowing.When first seen by us inMarch 2002, neurological exami-nation revealed marked ortho-static hypotension (supine: 150/90mm Hg; erect 3 minutes afterrising: 90/70 mm Hg), and slighthypomimia of the face, but nobradykinesia or hypokinesia ofthe extremities. His voice was softand monotone. Posture wasslightly stooped, but gait wasnormal. The arms displayed sub-tle and symmetrical signs ofrigidity, with cogwheeling. Therewas no resting tremor. The ret-ropulsion test showed mild pos-tural instability with a few stepsretropulsion, but unaided recov-ery. Vibration sense in the lowerlegs was absent, with a positiveRomberg sign. Tendon reflexeswere symmetrically brisk in boththe arms and legs, with Babinskiresponses in both feet. The pa-tient was diagnosed as having aprobable MSA of the striatonigralsubtype. In the following year, hedeveloped a marked, symmetricalbradykinesia and rigidity. Levo-dopa therapy was started and ti-trated to a maximal dose of 750mg/day, with an only minimalimprovement in his motor per-formance. He also receivedhydrocortisone to treat theorthostatic hypotension.Magnetic resonance imagingof the brain showed slight gen-eralized atrophy. [