A 76-year-old woman with a history of Parkinson’s disease (PD) for seventeen years, high blood pressure, atrial fibrillation, and stroke of the left anterior choroidal artery at the age of 68 years was initially referred for worsening headaches and hypothermia of 33.2 °C. Her medical treatment included amlodipine 5 mg, perindopril 8 mg, fluindione 10 mg, and 1 300 mg per day of a Levodopa Equivalent Dose. The hemodynamic state was stable, but the patient was confused, pale, and sweating. Brain MRI showed left anterior choroid stroke sequelae and significant vascular white matter hyperintensities without other abnormal findings (Fig. 1). EEG showed diffuse slowing without epileptic signals. The EKG was normal, notably without Obson’s J wave. Various blood tests were normal, as were cerebrospinal fluid tests. Furthermore, no environmental factors of hypothermia were noted. The resulting outcome was spontaneous and favorable, resolving within a few hours with only partial amnesia of the episode. The patient was subsequently rehospitalized three times within two months for similar episodes of headache with spontaneous hypothermia between 33 and 34 °C, with confusion and hyperhidrosis. During each hospitalization, clinical examinations including extensive infectious and endocrine studies were normal and the outcome was favorable, resolving within a few hours and sometimes associated with partial episode amnesia.
After experiencing similar recurring episodes, the patient was hospitalized again. During this hospitalization, four identical episodes were noted, each with a stable hemodynamic state and hypothermia between 32.5 and 34 °C (Fig. 2). The resulting outcome of each episode was spontaneous and favorable, resolving within few hours. During these episodes, no evidence of motor fluctuations of PD were noted. Assuming non-motor fluctuations (dysautonomic fluctuations) of PD to be the cause, 24-hour heart rate and blood pressure monitoring, (123)I-meta-iodobenzylguanidine (MIBG) cardiac scintigraphy, a levodopa challenge, and multiple orthostatic hypotension tests were performed. All exams were normal. Consistent with the hypothesis of Shapiro’s syndrome associated with PD, treatment with clonidine 0.15 mg twice daily was started. Following initiation of this treatment, the patient no longer experienced episodes of headache with spontaneous hypothermia over a follow-up of more than four years.