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Olivopontocerebellar Degeneration (OPCD).
1. Mental Health Consultation
Patient Name: B
Date: 2/22/99, 3/3/99
Identifying Data: Mr. B is a 69 year old, white, married father of three and retired
printer who was admitted to the B on September 5th 1995. He was referred for
evaluation by his attending physician Dr. K.
Reason for Referral: "during the past few months, E has been refusing care,
medications, treatments and labs… he has also become increasingly abusive towards
staff… his abusive behavior is both physical and verbal… he uses profanity and racial
slurs… during care he strikes out with fists and feet at staff… he has a history of
depression and is no longer taking his Paxil… in recent contacts he was un
communicative and made little eye contact.” Please evaluate and advise.
Background information:
• Has Olivopontocerebellar Degeneration (OPCD). Disease history and neuroimaging
were not available in his chart.
• All of his medications were discontinued on February 18th after he consistently
refused to take them. He had been taking Paxil 30 mg qd for depression, Primidone
for OPCD symptoms, Digoxin and Lopressor.
• He recently had a kidney stone removed. Prn Demerol and Vistaril were ordered on
2/18 for associated pain.
Mental Status Exam: He sits alone at a table in the pod near his room. When he eats he
spills food all over himself. He has a runny nose and seems oblivious to it. He is in a
constant state of motion due to spastic paralysis and cerebellar tremor (i.e. rocking back
and forth and twisting from side to side at the waste). His speech is profoundly
disarthritic and he speaks in a loud, booming, unmodulated voice. He responds to
questions in single words or very simple terse phrases. The answers to his questions belie
recent and remote memory impairment. He told me he worked for OTB all of his adult
life (He was in fact a printer and owned and operated S Press for many years). He said he
has four children when the chart indicates he has three children. He said his only leisure
activity was "my truck" when his history indicates he enjoyed many leisure time activities
including hunting, fishing, reading, painting and a working on his cottage. His demeanor
was calm, pleasant and cooperative. The he seemed bored and he looked out the window
fretting about the snow which might prevent his wife from visiting. He denied anxiety or
depression. Biological signs of depression were not present. His sensorium was clear.
He was alert and responsive. He was frustrated when intention tremor interfered with his
attempts to use gesture to augment his communications. He was impulsive and his insight
and judgment were both grossly impaired.
Diagnosis: Personality change due to neurological disorder, aggressive type
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2. Conclusions and Recommendations:
Given the absence of biological signs of depression and Mr. B's denial of depression,
I believe his negativism may be due to the progression of his disease rather than a sign of
worsening clinical depression. However, because of his history of depression and the
nature of his disorder, I certainly wouldn't rule out clinical depression.
Mr. B’s OPCD appears to be fairly well advanced and of the variety which includes
mild dementia. Comparing his current mental status, which includes disorientation and
memory impairment to his mental status at the time of admission to B in 1995, which
characterized him as free of cognitive impairment, suggests a rapidly progressing disease
process. Increasing anger, irritability and emotional liability would also be consistent
with this assessment.
Olivopontocerebellar Atrophy (OPCA) refers to a group of ataxias characterized by
progressive neurological degeneration affecting the cerebellum, the pons and the inferior
olives. Non-hereditary OPCA is a form of Multiple Systems Atrophy. There is wide
variation in the severity of symptoms and age of onset. The symptoms of OPCA differ
from person to person. Most patients experience difficulty with balance and coordination
of the legs and arms (ataxia) and dysarthria. Other symptoms may include hyperflexia
and Parkinsonisim (tremor of the hand or arm, rigidity, reduction or slowness of
movements). Loss of thinking and/or memory skills and difficulty controlling bladder or
bowels are common. Some forms of OPCA lead to dementia with prominent frontal
system dysfunction. Some patients have fatigue and/or sleep disturbance. Generally
symptoms of OPCA begin in mid-adult life. Onset is insidious and the course is
progressive, ending in death after about 15 years. Depression is common, but as can be
seen above, the Sxs of OPCA can also be mistaken for depression.
After reviewing the staff’s excellent behavior log, I have identified what I believe to be
some of the primary precipitants to Mr. B's aggressive outbursts. Each is listed below,
followed by a recommendation:
1. Visual hallucinations (i.e."The boy sitting on the dresser") probably hypnogogic in
nature (e.g. they occur in the early-morning or late evening hours) and perhaps caused
by the Primidone. Recommendation: place a night light in Mr. B's room, which is
bright enough to assist him with orientation when he awakes but not so bright that it
interferes with his sleep.
2. Frustration caused by his impaired ability to communicate with staff and other
residents. Recommendation: Be patient with him and allow him as much time as he
needs to complete his communications. The time invested in this up-front effort will
be more than offset by the time gained not struggling against his resistance to care.
3. Physical pain and the anticipation of physical pain associated with the provision of
care. Recommendation: Tell Mr. B you understand his concern about pain and
discomfort and if necessary offer prn pain medication prior to the provision of care.
Might consider ordering Darvocett for this purpose.
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3. 4. Shame and embarrassment related to incontinence, spasticity, dressing and eating.
Recommendation: Tell him that you understand his embarrassment and do whatever
it takes to help him maintain his dignity (i.e. male C N A for dressing, etc.).
5. Intrusions into his personal space by other residents (i.e. his seat in the hallway).
Recommendation: Try to head off these conflicts by steering other residents away
from his "special spots".
6. Impatience and concern that his basic needs might be overlooked (i.e. toileting with
staff assistance). Recommendation: Whenever you leave him on the toilet reassure
him that you will return promptly and check back with him frequently.
7. Reduced ability to modulate the tone, inflexion and volume of his speech secondary
to OPCD, which is misinterpreted by staff and other residents as aggression and
which sets into motion a subtle and inadvertent self-fulfilling prophecy.
Recommendation: explain this neurological deficit to staff and when appropriate to
other residents.
Additional Recommendations:
1. Ask Mrs. B to visit according to scheduled times and provide Mr. B with a schedule
of his wife's visits.
2. Ask the activities folks to provide Mr. B with simple failure free activities, which can
keep him, occupied while he is sitting in his “special spot “on a unit.
3. If Mr. B is willing, at any time in the near future, I suggest a trial on Remeron. Also,
considering the similarities between Mr. B's clinical presentation and that of the
resident K.P., he might benefit from Risperdal if he becomes acutely agitated. He
would either benefit or it would worsen his symptoms. In any case, it's not indicated
now.
___________________________
Drew Chenelly, Psy.D. Date:
Clinical Neuropsychologist
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