| Case 10 2/22/99 Disorders of higher function |
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| Case discussion Mr. X is a 70-year-old retired college professor. who is accompanied by his wife. Mrs. X is concerned about her husband’s memory. When you question her about her precise concerns, she notes that he will frequently walk into a room and forget why he has gone there. In addition his ability to recall things that have happened to him recently is diminishing. For example, his son and daughter-in-law recently visited from the East coast, and a few days afterward he asked his wife when the were coming to visit. On one occasion he went to the store to pick up a few grocery items and returned 3 hours later, unable to account for why he was gone so long. Mrs. X cannot precisely date the time she became concerned about her husband, but believes that the problems are getting worse. She thinks he may be depressed because he is less interested in spending time with friends and is losing interest in reading and going to the movies. Mrs. X himself feels his wife is overreacting. He says that he has no concerns about his memory and that he feels fine. He is still able to manage the household finances, play golf, and work in his garden. He is able to manage all his activities of daily living. He denies feelings of sadness, sleeps well, and has a good appetite. Mr. X has an unremarkable past history. He had an appendectomy many years ago and has had mild untreated hypertension. He never smoked, and drinks about 2 drinks of hard liquor each evening. His wife is concerned that he drinks too much. He is taking no medications. His mother died at age 72 of a stroke, his father died at age 57 of a myocardial infarction. One brother is alive and well and is 75 years old. On examination, he has a BP of 145/90, pulse 76, and appears in good health. He is alert, knows he is in a doctor’s office but did not recall the exact address or your name. He gives the date as Tuesday, January 25, 1998 when it is in fact Tuesday, January 26, 1999. He knows that the current president is Bill Clinton, and can tell you that he was recently impeached by the House because of a "sex scandal." He can recite the presidents in reverse order back to FDR. He cannot recall any of 4 words you have given him to remember after 5 minutes, and is not quite sure that you have even given him any to memorize. His spontaneous speech is fluent, with normal comprehension, and repetition. He has some difficulty naming unusual objects — although he named a watch and pen well, he called the watch buckle "a thing for closing it" and was unable to generate the name for a shoelace. He is able to follow a three-step command. He draws simple geometric figures well. Examination of the cranial nerves, motor and sensory system are normal. Although his deep tendon reflexes are generally normal, you cannot elicit ankle jerk reflexes. There is no Babinski sign. Cerebellar testing and gait are normal. Pathologic Case Correlate: Neuropath Case 10 |
A 70 year old man presents with slowly progressive anterograde memory dysfunction and possible depression. Physical exam revealed disorientation to time, confusion, no 5 minute recall, difficulty naming objects. However, cranial nerve, motor, sensory, and cerebellar exam were all normal. what is the time course? Chronic, slowly progreesive disease: has degenerative or metabolic etiology good, lesion is likely to be in the hippocampus; note involvement of hippocampal, dorsal medial nucleus limbic circuitry in thiamine deficiency tends to cause confabulation, not seen here. Inability to form recent memory and agnosia, impairment is in the following circuit: temporal and parietal association tracts that feed both the hippocampus and dorsal medial nucleus via the parahippocampal gyrus. good
Alzheimer’s disease I do not accept normal aging as a cause of the patient's memory loss. Depression is a good alternative possiblity. CT / MRI one would see brain atrophy, possibly enlargement of ventricles Stop EtOH and any OTC drugs memory would improve if EtOH or the OTC drugs were causing the impairment. clinical course; evaluation for treatable causes of dementia esp B12 deficiency would possibly refute your hypothesis Progressive disease with no cure. Adminstering periodic mini MSE’s that are scored will reveal deterioration. complications include: aphasias, apraxias, agnosias, memory loss, loss of judgement, inability to care for self, behavioral problems, increased tendency towards acute confusional states, injuries, getting lost,incontinence, pneumonias and other infections
The underlying pathology associated with Alzheimer’s dementia is a loss of
synapses where the magnitude of synapse loss correlates with the severity of
dementia. Synapses in the hippocampus are depleted early paralleling the
progressive decline in memory function. There is also damage to cholinergic
nuclei in the basal forebrain and acetyl choline depletion in the cortex and
hippocampus. Other pathology includes neuritic plaques, (amyloid deposition),
in the hippocampus and parietal cortex as well as neurofibrillary tangles
(helical filaments formed by excessive phosphorylation of tau protein),
within
pyramidal cells of the cortex. ok; neuritic plaques are abnormal synapses; amyloid deposition represents the deposition of what protein in the synaptic cleft? How does this protein deposit or precipitate in familial Alzheimer's disease. Are the systems involved in AD limited to the cholinergic ones? Providing support for the caretaker often a spouse is a critical part of the treatment of a patient with AD. This support should involve the counseling of the family as you say especially any adult children. |
This is a 70 year-old retired professor whose wife describes an insidious loss of recent memory, anhedonia, anomia, and excessive alcohol intake. The duration of symptoms is not given. The patient denies that any problem exists. it is probably more correct to say the duration of symptoms is unclear rather than to say it is not given. good summary. Loss of short-term memory: hippocampus, limbic system, neocortex good We're not given the clinical course, but the most likely course is a slowly progressive, degenerative course (assuming patient's wife would have noticed a sudden onset) youre the physician taking the hx; you asked the spouse for the time course; she "cannot precisely date the time she became concerned" when you cannot get the time course it is dangerous to assume one. Alzheimer's dementia, early good
- Other degenerative dementias: Pick's disease, Lewy Body Dementia,
normal-pressure hydrocephalus, progressive supranuclear palsy. good
- CT or MRI: diffuse cortical atrophy, atrophy of hippocampi, enlargement
of ventricles: all of these are suggestive but not diagnostic of AD good; an autopsy may be important in confirming the dx postmortem if you are concerned about the familial form of the disease for instance to help with counseling.
- disability due to declining mental function -- decline in Folstein MMS
score, follow-up care. This includes alteration in sleep cycles, hostility
and aggression, inability to carry out activities of daily living, becoming
a burden on family members. good
The pathophysiology of Alzheimer's Disease (AD) is thought to involve loss
of neurons starting in the hippocampus, leading to the early onset of
memory impairment , with gradual involvement of other parts of the brain,
especially the entorhinal cortex, temporal cortex, and parietal cortex,
with concomitant progressive loss of cognitive function. AD has been
called a "disconnecting syndrome", because although the sensory and motor
functions are largely intact, integration of sensations, thought, memories,
and actions is impaired, almost as if each part of the brain were
disconnected from the rest. The reason for the loss of neurons is unknown,
but several suggestive associations have been found. First, Down's
syndrome patients, who have an extra chromosome 21, tend to die early in
life with dementia and postmortem brain changes indistinguishable from AD.
This is thought to be due to overexpression of a protein found of
chromosome 21, amyloid precursor protein (APP). Accumulations of the
product of APP, amyloid-beta, are found in brains of AD patients and Down's
patients at autopsy (amyloid-beta form the major component of senile
plaques). This line of evidence suggests that abnormal accumulation of
amyloid-beta in AD may be associated with the dementia; however, the amount
of amyloid-beta accumulation does not correlate with clinical severity of
disease in many cases. Another association that has been found in AD is a
genetic predisposition in several forms of the disease. The epsilon-4
alleles of Apolipoprotein E gene tend to confer greater risk of developing
AD at an early age. Apolipopritein E regulates lipid metabolism, and has
been implicated in the pathogenesis of senile plaques. Finally, another
pathologic sign of AD at autopsy, neurofibrillary tangles, are cytoplasmic
inclusions within neurons which are composed of aggregates of abnormally
phosphorylated tau protein, normally a component of the cytoskeleton.
Again, the significance of this is not known.
very good points: too long however |