Annals of Long Term Care

Author, Discussant, and Attendee Affiliations: Cleveland Clinic, OH (Hope Torregosa, MD, and Amanda Lathia, MD); Community Care for the Elderly, Milwaukee, WI (Linda Culhane, GNP, and Priscilla Sharpless, CAPSW); Johns Hopkins University School of Medicine, Baltimore, MD (Ashley Hicks, MD, and Jessica Golin, MD); Madison VA Hospital, Madison, WI (Timothy Howell, MD); Mayo Clinic, Rochester, MN (Aimee Yu-Ballard, MD, and Bernard Aoun, MD); University of Texas Health Science Center, San Antonio (Liliana Oakes, MD); University of Texas Southwestern Medical Center, Dallas (Vivyenne M.L. Roche, MD, and Sarwat Jabeen, MD); and University of Wisconsin School of Medicine and Public Health, Milwaukee (Michael Malone, MD, Ejus Culleth, MD, and Julie Luks, MD).

Case Presentation 

A 73-year-old white woman with a history of depression for more than 30 years visited the clinic with her son and daughter for a comprehensive geriatric assessment. The patient’s family was concerned about her deteriorating mental state and confusing medical diagnoses, especially over the previous 5 years. Without access to her medical records until recently, and because the patient had a tendency to keep her health problems to herself, the children were unsure about certain specific information regarding their mother’s health. Even when her cognition was clear, the patient did not want to reveal much information to her children or to her husband. Her children were, however, able to access some of her medical records.

Relevant History

As a child, the patient lived in Poland and was in hiding with her family for 8 years during the Holocaust. She received a depression diagnosis after her first husband left her in the 1970s, but she remained functional, managed a business, and took care of her children despite her diagnosis; therefore, her children remained unaware of her diagnosis at that time. The patient’s daughter reported that her mother’s mood again deteriorated when the youngest child got married and moved out of the family home. She was treated with lithium and other unknown psychiatric medications in the 1980s for depression and may have had a diagnosis of bipolar disorder. She still remained fully functional, however, remarried in 1991, and continued to run a successful business. Her second husband was initially unaware that she had been treated for depression and had received psychiatric care.

According to her daughter, the patient remained on lithium until around 2005. She recalled a situation when the patient visited her that year looking “dopey and drugged.” At that time, the patient was taking lithium, trazodone, alprazolam, and an unrecalled antidepressant after having had several medication changes, including stopping lithium for a period of time. The patient’s children were aware that she was seeing a psychiatrist around that time, but they did not know how often. However, they noted a dramatic decline in her cognitive and functional abilities and decided that she should stop seeing her current psychiatrist and seek a second opinion from another psychiatrist. At her first visit with her new psychiatrist in 2005, a workup was initiated, and a magnetic resonance imaging (MRI) scan showed the following: intact orbits and retro-orbital structures; an asymmetric rightward gaze to the right lobe; a mildly hypertrophic pituitary gland; no intracranial mass or mass effect; no hydrocephalus; and age-related involutional changes, which included subtle ex vacuo enlargement of the subarachnoid spaces and ventricular system with confluent regions of T2 prolongation within the subcortical white matter of both frontoparietal junctions and, less substantially, within the temporal lobes, compatible with chronic microangiopathic gliosis.

The patient’s daughter was told that her mother demonstrated some mild impairment on cognitive testing. Her family informed the psychiatrist that they noticed the patient had some memory loss. They recalled that she had gotten lost on occasion while driving starting a few years before the initial assessment with the second psychiatrist. The patient was started on donepezil and memantine, taking them concomitantly or separately for short time periods, but they were discontinued at an unknown time after the patient showed no improvement. At the time of her first visit with her new psychiatrist, she was taking scheduled alprazolam and trazodone for sleep, but these were changed to quetiapine, as-needed alprazolam, sertraline, and as-needed lorazepam. The chart review noted “bipolar disorder.”

In 2007, the patient was hospitalized twice at a psychiatric unit for depression and anxiety. Around that time, she began experiencing difficulty initiating movements, slowing of movements, and tremors. There was also a situation at her daughter’s birthday party when she complained that she could not get up from her chair. She was referred to an orthopedic surgeon, a neurosurgeon, and, subsequently, a neurologist. She also changed psychiatrists again. MRI scans of the spine showed multilevel degenerative disc disease, which occurred after her inpatient psychiatric hospitalization.

Her neurologist noted further slowing of movements in July 2008 at a regular follow-up appointment. The patient informed him that she had difficulty initiating ambulation and that her handwriting had become smaller over the previous 2 to 3 years. She was drooling, but had no difficulty chewing or swallowing. Her neurologist noticed that her voice had become softer and lower. She informed him that she experienced difficulty getting out of a car for the previous 6 months and that she suffered from back pain, joint stiffness, and chronic constipation. She had no history of syncope, fainting, or seizures. Her memory was mildly impaired. She and her family were unclear as to whether she had visual or auditory hallucinations. She was experiencing no diplopia or vision changes. At the time of this visit with the neurologist, her medications were quetiapine, escitalopram, mirtazapine, omeprazole, and polyethylene glycol, and it is unknown when these agents had been initiated. On physical examination, she had masked facies, a slow gait without festination, mild bradykinesia, slowed fine finger movement, a mild resting tremor without pill rolling, and cogwheel rigidity. The electroencephalogram results were normal. Somatosensory evoked potentials were consistent with sensory polyneuropathy or lumbar radiculopathies. The patient received a diagnosis of parkinsonism superimposed on lumbar and cervical spondylosis. A trial of carbidopa, levodopa, and entacapone was initiated. The rest of her medications (quetiapine, escitalopram, mirtazapine, omeprazole, and polyethylene glycol) were continued.

One month later at a follow-up visit, it was noted that the patient’s bradykinesia had improved; however, she scored 22 of 30 on the Mini-Mental State Examination (MMSE), and the results of the Clock Drawing Test were abnormal. Her neurologist discussed the diagnosis of parkinsonism and cognitive impairment with the patient’s husband and daughter. Carbidopa-levodopa was started and carbidopa-levodopa-entacapone was stopped, as the latter had caused diarrhea. Physical therapy was recommended, but the patient did not follow through, despite urgings from her family.

On subsequent visits to the neurologist over the next year, the patient’s parkinsonian symptoms continued to improve. Her main symptoms were micrographia, tremors, constipation, and difficulty buttoning her shirt and standing up from a seated position. Ropinirole and propranolol were added to her medication regimen, despite concerns over her history of extreme sensitivity to numerous medications. She could no longer manage her finances or shop alone, and she required assistance leaving the house. The patient’s children became aware that she began having difficulty preparing meals and keeping track of current events and television shows; however, her husband, who lived with her, denied these reports to her children and to her doctors.

In November 2009, her family decided to seek a second opinion and consulted with another neurologist, as they felt that her current neurologist did not communicate well with her psychiatrist, whom she was seeing regularly. Her new neurologist noted that the patient had significant tremors, severe bradykinesia and rigidity, postural instability, and dysphonia. Her examination showed masked facies, axial rigidity, bradykinesia, resting tremor, cogwheel rigidity (left greater than right), festinant wide-based gait, retropulsion/anteropulsion, and postural instability. Her neurologist made the diagnosis of severe Parkinson’s disease of relatively recent onset. Carbidopa-levodopa 25/100 mg was increased to 1.5 tablets three times daily, and ropinirole XL 2 mg every evening was continued. Home physical therapy and gait training were again suggested, and a workup was restarted.

On NeuroTrax testing, her cognitive performance was 1 standard deviation (SD) below the average in memory, executive function, attention, and visual/spatial. Her global cognitive function performance was 1 SD below the average. The complete blood count, metabolic panel, thyroid panel, vitamin B₁₂ levels, folate levels, rapid plasma regain screening test, and fluorescent treponemal antibody absorption test were all normal. A brain MRI scan revealed stable areas of chronic ischemic demyelination in the subcortical deep white matter, generalized atrophy of the brain parenchyma, a prominent pituitary gland, and a mucous retention cyst in the left maxillary sinus. The patient’s brain parenchymal signal was normal, without any mass, mass effect, ischemia, or abnormal extra-axial fluid collection.

Regular clinic visits to her neurologist and psychiatrist from January 2010 to September 2010 resulted in several medication changes. The rivastigmine patch was added, initially at 4.6 mg every 24 hours, then increased to two patches, and later changed to 9.5 mg every 24 hours. Ropinirole was increased to 1 mg three times daily, with ropinirole XL 2 mg given at night. Escitalopram was decreased from 30 mg to 20 mg daily, and she was started on scheduled clonazepam, quetiapine, and lorazepam, as needed. Clinically, the patient was still manifesting signs of memory loss, dementia, anxiety, and depression. She was restarted on memantine by her primary care doctor.

By March 2010, she had become dependent in activities of daily living (ADLs) and instrumental ADLs (IADLs) and required caregiver assistance 24 hours a day 7 days a week, which was paid for by her children. Around this time, the patient’s children noted that she was having hallucinations and delusions, including believing that her husband was having affairs with the caregivers. Her husband had become very stressed about his wife’s situation and was deteriorating physically. Her children suspected that he was verbally abusing her. After visiting with the neurologist in September 2010, the family decided to move the patient from her home into an assisted living facility near one of her sons, who lived in a different state. The patient’s husband was amenable to this arrangement, and the children acquired power of attorney. Her daughter said that the patient’s psychiatrist had declared her incapable of making her own decisions. At this time, she was advised to stop the as-needed lorazepam, and rasagiline was added to her medication regimen due to her worsening parkinsonian symptoms.

The facility provided meals, ADL and IADL assistance, and medication administration. The patient did not have an around-the-clock companion, as her husband remained at home; however, her family was willing to hire someone to fill this role, if needed.

After the patient moved to the assisted living facility, her children were able to observe her more closely. They noted regular fluctuations in her mental status throughout the day that appeared to coincide with her carbidopa-levodopa dosing, which was still being administered three times daily. They discovered that she was often lucid and alert in the mornings, but became confused and appeared “spaced out” by lunchtime. She then became more alert after her midday carbidopa-levodopa dose, only to become more confused later in the afternoon, followed by another period of alertness in the evening. Her children also observed that her appetite coincided with her mental state. She ate a very good breakfast, very little lunch during her period of declined cognition, and a good dinner as she became more alert. The patient’s children described their mother as severely anxious. Whenever the patient’s husband came to visit her, she became very agitated, and she told her children that he was part of the problem.

Current Presentation

On the morning of October 2010, 1 month after the patient was admitted to the assisted living facility, she was seen in a movement disorders clinic. A diagnosis of a parkinsonian disorder and dementia was made; rasagiline was discontinued. She was noted to be “very alert, pleasant, and cooperative.” That afternoon, she was seen at the Cleveland Clinic in our geriatrics clinic for the first time per her family’s request. She was confused, barely able to follow commands, and was moaning. Her family provided her history and whatever medical records they were able to obtain.

A review of systems revealed a significant history of weight loss (5-20 lb over the previous 6 months and approximately 20 lb over the previous 5 years) and constipation. The patient had been reporting constipation and abdominal pain for at least 5 to 7 years prior to her current presentation. She experienced pain in her left upper quadrant after eating, which was associated with nausea and occasional vomiting and was relieved by having a bowel movement. She had two colonoscopies and a polypectomy in 2005; the polypectomy showed benign lesions but no other abnormalities. A computed tomography scan of the abdomen and pelvis in March 2009 showed a benign Bosniak category II renal cyst. She had never had an upper endoscopy examination. Her constipation was very severe at times, with more than one episode of impaction. She manually disimpacted herself on occasion. She had been treated with omeprazole and scheduled psyllium at some point for her gastrointestinal problems. Her last normal bowel movement had been over 1 week prior to her current presentation.

During her physical examination at the Cleveland Clinic, she was sitting in a wheelchair. We noted that she was well nourished, kempt, diaphoretic, and anxious. Her vital signs were within normal limits. The patient’s head, eyes, ears, nose, and throat were examined. Her head was normocephalic/atraumatic. Her tympanic membranes could not be viewed due to soft cerumen in the external auditory canals. The patient’s neck was supple, her trachea was midline, her thyroid was not enlarged, and there was no cervical lymphadenopathy. Her respiratory system was clear on auscultation bilaterally, with no rales or wheezing observed. Her cardiovascular examination revealed a regular rate and rhythm; normal S1 and S2 heart sounds; and no murmurs, rubs, or gallops. Her gastrointestinal examination was positive for bowel sounds on auscultation; her abdomen was soft and mildly tender diffusely, and there was no hepatosplenomegaly. On her extremities, we noted minimal grade 1 bipedal edema, but no clubbing or cyanosis, and 2+ bilateral dorsalis pedis pulses. There was no evidence of skin breakdown or rashes.

The patient was unable to stand without assistance on the Get Up and Go Test. Her upper extremities demonstrated four-fifths strength. She then stopped following directions, so lower-limb strength, finger-to-nose testing, heel-to-shin testing, rapid alternating movements, and fine finger testing were unable to be assessed. The patient moved her extremities spontaneously, and a resting, pill-rolling tremor of her hands and left leg were noted. She also demonstrated cogwheel rigidity greater on the left side than the right and masked facies. Cranial nerves II through XII were intact. A sensory examination showed a normal light touch. On the MMSE and the Geriatric Depression Scale (GDS), she scored 12 of 30 and 3 of 15, respectively. For her Clock Drawing Test, she drew three short lines inside a circle. The patient appeared to be very confused and continued to be unable to follow directions.

Her current medications were omeprazole 40 mg orally per day; rivastigmine patch 9.5 mg every 24 hours; mirtazapine 30 mg at bedtime; escitalopram 20 mg orally per day; carbidopa-levodopa 25/100 mg, 1.5 tablets three times daily; ropinirole XL 2 mg at bedtime; ropinirole 1 mg twice daily; memantine 10 mg twice daily; clonazepam 0.5 mg, one tablet in the morning, one-half tablet at lunch, one-half tablet at 4 pm; vitamin D 1000 IU daily; vitamin A/C/E combination, one tablet daily; multivitamin, one tablet daily; and psyllium, two tablets twice daily.