D ementia with Lewy bodies (DLB) is characterized by a triad of core features: fluctuating cognition, visual hallucinations, and parkinsonism; other commonly associated features include REM sleep behavior disorder (RBD) and dysautonomia (e.g., orthostatic hypotension, urinary incontinence, constipation, and impotence).1 Case-control studies have reported a higher prevalence of rhinorrhea in patients with Parkinson disease (PD) compared to healthy controls.2-6 Of interest, 76% of patients with PD reported rhinorrhea exacerbation while eating.2 Further studies have corroborated a 5-fold increased prevalence of rhinorrhea in patients with PD compared to controls and also an association with lightheadedness among rhinorrhea-affected patients with PD. 4 We report a patient with clinically probable DLB who presented with activity-dependent rhinorrhea consistently followed by hypotensive syncope, suggesting that the rhinorrheahypotension association may be temporally closer than previously suspected, and highlighting the value of nonpharmacologic therapeutic strategies in this clinical situation.
Case reportAn 85-year-old Ashkenazi Jewish man, type 1 N370S Gaucher disease (GBA) carrier, with history of peripheral neuropathy, hyperlipidemia, and coronary artery bypass grafting, complicated by hypotension at age 74, and dream enactment behaviors suggestive of RBD, presented with a 5-year history of tremorless parkinsonism with visual hallucinations and fluctuating cognition at symptom onset, followed by orthostatic hypotension, meeting McKeith criteria for clinically probable DLB.1 The patient denied a history of anosmia, diabetes, allergies, nasal, or sinus complaints. Eating, urinating, and defecating consistently induced rhinorrhea, followed by hypotensive syncope within minutes. Blood pressure (BP) during these events was documented to be as low as 60/40 mm Hg. However, his BP markedly fluctuated throughout the day, with readings as high as 180/100 in the sitting position.Trials of nasal spray were futile. Carbidopa/levodopa exacerbated the hypotensive syncope and hallucinations and thus was discontinued. He refused separate trials of rivastigmine and quetiapine. To exclude CSF as the source of rhinorrhea, given that some triggering activities were associated with Valsalva-type actions, the patient's nasal discharge was examined for b-2 transferrin, which was negative. None of the patient's medications was known to have previously induced rhinorrhea and syncope.The family referred to the sequential events of rhinorrhea and hypotensive syncope as the runny nose sign (video at Neurology.org/cp), and considered it his most disabling feature. Efforts at optimizing the dosage of fludrocortisone and midodrine did not reduce the frequency of these episodes. The patient would secrete copious clear, thick nasal discharge, invariably announcing hypotensive syncope within minutes. Rhinorrhea would continue
Practical ImplicationsConsider administering a vasopressor prior to activities that generate syncope and substantial disabi...