Responsiveness of dysphagia to acute l-Dopa challenge in progressive supranuclear palsy

Abstract

Dysphagia occurs in up to 80 % of patients with progressive supranuclear palsy (PSP) [1], and may result in aspiration pneumonia, which is the leading cause of death in PSP [2]. We report the improvement of dysphagia following L-Dopa challenge in three patients diagnosed with probable PSP [3] and evaluated for enteral nutrition. All patients showed MRI signs suggestive of PSP [4] and had the specific finger tap pattern of ‘‘hypokinesia without decrement’’ [5]. None of them had prior or concomitant history of REM Sleep Behaviour Disorder. Disease severity was rated through the Progressive Supranuclear Palsy Rating Scale and Staging System (PSPRS) [6]. Dysphagia was evaluated by a trained Speech and Language Therapist through the Dysphagia Rating Scale (DRS), a recently developed tool able to predict the risk of complications of dysphagia [7] (Table 1). Case 1: 78-year-old male with PSP history of 5 years, treated with L-Dopa 300 mg/day. He showed complete supranuclear upward and downward gaze palsy, increased latency onset of horizontal saccades, square wave jerks, positive applause sign, moderate dysarthria, moderate rigidity in the upper and lower limbs, mild dystonia and akinesia in the right arm and hand, trunk apraxia and inability to walk unassisted. Mild oral apraxia associated with oromandibular dystonia resulted in prolonged oropharyngeal phase, food accumulation, and coughing. L-Dopa was increased up to 600 mg in 2 days, leading to less frequent episodes of oral apraxia and decreased latency of the oropharyngeal phase, with significant reduction of dysphagia risks. He received logopedic training, and for the following 3 months his condition was stable. Case 2: 66-year-old male diagnosed with PSP at the age of 61 following the onset of gaze abnormalities, tremor, falls, and bradykinesia and treated with low doses of L-Dopa to mild benefit. At the time of L-Dopa challenge he showed complete vertical and partial horizontal supranuclear gaze palsy, positive applause sign, severe dysarthria, neck dystonia, severe axial and limb rigidity, dystonia, and akinesia of the right hand. He was wheelchair-bound and unable to stand unassisted. Prolonged oropharyngeal phase due to oral dystonia and oral apraxia was observed. L-Dopa was increased up to 1,200 mg/day, with improvement of swallowing. He received logopedic training, and at 6 months’ follow-up his condition was stable. Case 3: 69-year-old female with a PSP history of 4 years, treated with L-Dopa 400 mg/day. She came to our observation after progressive worsening of her general conditions such as severe axial and limb rigidity, severe dystonia and akinesia in her left arm and leg, akinetic mutism, complete vertical supranuclear gaze palsy, and severe oral apraxia and oromandibular dystonia that made her almost unable to open her mouth or push food behind. She showed signs of malnutrition and dehydration. L-Dopa was increased to 1,000 mg in 4 days. By the second day, the patient showed a reduction of body stiffness and spontaneous mouth opening at food presentation. After a S. Varanese (&) P. Di Ruscio L. Ben M’ Barek Casa di cura Villa Pini d’Abruzzo, Policlinico Abano Terme, Via dei Frentani 124, 66100 Chieti, Italy e-mail: sara78_96@yahoo.it