22.1 Clinical History – Main Complaint

This 62-year-old former accountant presented to a neurologist with complaints of a right hand tremor, slowness, and gait difficulties. He was diagnosed with Parkinson’s disease (PD) and received pramipexole up to 1.5 mg tid. On this medication, he reported being well for 2 years. After 3 years of evolution, levodopa/carbidopa was introduced because of a worsening of his parkinsonism. His tremor was initially significantly improved by levodopa, but the effect was lost after less than a year. Higher doses caused episodes of hypotension and also spasms of his neck and toes which were treated with clonazepam. At the fifth year, he developed significant freezing of gait and needed a walker to ambulate safely.

The patient never seemed to have motor fluctuations or end-of-dose wearing off. In addition, he complained of a muffled voice, dry mouth, nycturia at least three times per night, incontinence at times, and dream enactment. He admitted having erectile dysfunction for some years before his diagnosis. Recently, he was reported not to participate in social gatherings as he had before, and he seemed not to have interest in any activities be it at home or outside. He had been diagnosed with a depressive disorder for which he was taking citalopram. All his symptoms lead to a gradual loss of autonomy. He was eventually sent to a movement disorder clinic for medication optimization.

Upon further questioning both him and his wife, it was found that he had left to his wife most of his household responsibilities including the finances, a significant factor for an accountant. Although it was not initially clear whether this was due to his apathy or to cognitive deficits, it became clear that he had significant cognitive impairments including not being able to be trusted to take his medications and to keep track of his appointments. His wife described a generalized disorganization. He had to retire earlier than he wished because he felt he did not have the energy to follow the accounts of his clientele.

22.2 General History

The patient was married and the couple had two healthy children. His past medical history was remarkable for a von Willebrand disease and two discal hernias. Years ago, he also had a cholecystectomy. His medication consisted of tamsulosin, clonazepam, citalopram, sildenafil, baclofen, pramipexole (1.5 mg TID), and levodopa/carbidopa 100/25 six times per day.

22.3 Family History

Both his parents died of heart disease in their early seventh decade. The patient had an older brother who had had a coronary artery bypass at age 57 and an older sister who died of breast cancer at age 66. There was no history of PD or other neurological disease in the family.

22.4 Examination

Vital signs were notable for orthostatic hypotension with a drop of systolic blood pressure of more than 30 mmHg without tachycardia on multiple measuring. Blood pressure was otherwise at 105/70. Heart rate was unremarkable. Cardiopulmonary exam was normal.

The patient was alert and cooperating well. Bedside cognitive testing revealed a mild disorientation in time, general slowing of thought processes, diminished verbal fluency, recall difficulties that were improved by cues, and distractibility. He also had impairments on figure drawing and copying. His score on the Montreal Cognitive Assessment (MoCA) was 21/30, and the test was fairly long to pass. On the clock drawing, the numbers were spaced unevenly and included supplementary numbers.

Cranial nerve examination revealed slow, jerky ocular pursuit but no gaze limitations or saccade anomalies. The patient had hypomimia, dysphagia while attempting to drink out of a glass of water, and a dysphonia with a peculiar quivering voice and an increased pitch.

Parkinsonism was present, heralded by symmetrical mild to moderate rigidity and bradykinesia of the upper and lower limbs. A slightly asymmetrical jerky postural and action tremor of the upper limbs was noted. It nearly disappeared at rest. Gait was notable for a stooped posture, slowness, occasional freezing, decomposed half-turn, and diminished arm swing. Although the patient did not fall, he was unstable without his cane and showed retropulsion. Examiners were not able to note any motor fluctuations throughout the interview even though the patient was near the time of his next levodopa dose.

Strength was normal. There were no sensory impairments or ataxia. The stretch muscle reflexes were increased at 3/4 all over and the plantar responses showed Babinski signs.