Neurology: Case of the Month

History and Physical — Patient 44

History

Present Illness: The patient is an 81 year old white female who was referred for re-evaluation of cognitive status. She has a history of memory dysfunction that was first noted in late 1998. She initially had problems getting lost while driving in familiar places. She was also noted to have difficulties with object naming, word-finding, and persistence on tasks. Her family reported a change in her personality. In this regard, she was felt to be somewhat more child-like and abrasive toward other people. She would check her purse multiple times to see if she had any money, and would get excited very easily. A neurologist evaluated the patient in December 1998. MRI of the brain was performed, and was reported to show non-specific white-matter changes and age-related cortical atrophy. A SPECT scan demonstrated reduced perfusion in the frontal and temporal lobes bilaterally. Neuropsychological testing showed significant memory impairment, with preservation of attention/concentration, visuoperceptual functions, and language. She was given a diagnosis of early Alzheimer's Disease and was started on Aricept, Zoloft, Vitamin C and Vitamin E. The family reported that her behavioral problems improved after starting these medications, but the memory problems have remained unchanged. The patient was subsequently evaluated in a tertiary referral center in July 1999, for a second opinion regarding her cognitive problems. Neuropsychological testing was repeated, and again the pattern of severe memory impairment with preserved attention, visuospatial and language functions were noted. However, mild executive dysfunction, motor disinhibition and impaired motor programming were also noted. This led to a diagnosis of possible frontal-lobe dementia.

There is no history of head trauma, nonprescription drug or alternative medicine use, depressed mood, appetite changes, early morning awakening, or altered temperature sensitivity. The patient has gained weight over the past several years.

Current Status: The patient continues to have significant problems with her memory, and relies on a full-time caregiver for support. She is able to feed herself, and dresses herself with assistance. She does not drive, and she relies on her son for assistance with management of her financial affairs. She states that she can still perform arithmetical operations, and write her own checks. She is hard of hearing, and her visual acuity has declined since her previous assessment. This is due to macular degeneration, which has required treatment with bilateral laser ablation surgeries. The caregiver has noted some episodes of child-like behavior, and lack of initiation of daily activities such as tooth brushing and bathing.

Past Medical History: Rheumatoid arthritis, diabetes mellitus (controlled with an oral hypoglycemic agent), cardiac arrhythmia (tachyarrhythmia, presumed atrial flutter, controlled with digoxin), macular degeneration bilaterally, transient right-sided facial droop (early 1999), which spontaneously resolved.

Past Surgical History: Bilateral cataract removal with lens implants in 1993, surgical removal of multiple benign skin lesions, laser ablation surgery (October 1999, January 2000).

Medications: Vitamin E 1000 IU b.i.d., donepezil 10 mg q.hs, sertraline 50 mg q.am, Vitamin C 500 mg b.i.d., propoxyphene/acetaminophen 1 tab q.6 hrs p.r.n. pain, glimepiride 1mg q.d., digoxin 0.25mg q.d. (except Sundays), multivitamin, naprosyn 220 mg b.i.d.

Family History: Sister with an unknown dementing illness, brother with coronary artery disease and a history of myocardial infarction.

Social History: Lives with son, daughter-in-law, and grandchild. She has a full-time attendant/caregiver as above. She reportedly drinks 1-2 glasses of wine per week. There is a remote history of tobacco use.

Review of Systems: There is no history of sudden visual loss, headaches, chest pain, dyspnea, urinary symptoms, gynecological symptoms, or dermatologic problems.

Physical Exam

Vital Signs: B.P. 144/90; pulse 68; temperature 98.2 F; respiration 16.

HEENT: Normocephalic/atraumatic; pupils equal, round and reactive to light and accommodation, oropharynx/nasopharynx clear, mucous membranes moist.

Neck: Supple without lymphadenopathy, no carotid bruit.

Chest: Lung fields clear to auscultation bilaterally.

Cardiovascular: Regular rate and rhythm, II/VI systolic ejection murmur loudest at the left upper sternal border.

Abdomen: Soft, non-tender and not distended, normal bowel sounds present.

Extremities: No clubbing, cyanosis or edema, peripheral pulses present bilaterally.

Neurological Examination

Mental Status: Alert, oriented to name, place, and year, but not to month, date or day of the week. Initial registration of three items was intact, but she was unable to recall any of the items after three minutes. She lost two points on serial seven testing. Naming, repetition, and comprehension of a three-step command were intact. Reading, sentence writing and drawing were also intact. No perseveration was noted during the testing. (MMSE = 22/30).

Cranial Nerves:

Cranial Nerves

Findings

INot tested.
IINo afferent pupillary defect, pupils equal, round and reactive to light. Visual fields intact peripherally; central visual acuity 20/200 OU.
III / IV / VIExtra-ocular movements were intact without nystagmus or diplopia. No diminution of vertical gaze was observed.
VTemporalis and masseter intact bilaterally; sensation symmetrical over the entire face
VIIFacial movements symmetrical.
VIIIHearing acuity decreased bilaterally.
IX / XGag intact, uvula in midline.
XISternocleidomastoid and trapezius intact bilaterally.
XIITongue protruded in midline; no fibrilations or atrophy.

 

Motor: Bulk and tone was normal, Neck flexors and extensors 5/5; Upper extremity strength 4/5 proximally, 5/5 distally; lower extremities: 4/5 proximally, 5/5 distally.

Reflexes: Triceps 1+ bilaterally, biceps 2+, brachioradialis 2+, patellar 1, ankle 0; Hoffman, jaw jerk, snout, and palmomental responses negative, glabellar response mildly positive.

Sensation: Intact to pinprick, temperature, light touch, vibration and proprioception bilaterally; Romberg was negative.

Coordination: Intact to finger-to-nose, heel-to-shin, and rapid alternating movements. No truncal ataxia, ocular dysmetria, or loss of ability to check flexion movements.

Gait: Slightly wide-based, but normal arm swing and stride. Able to perform heel and toe walking with difficulty, but unable to perform tandem walk.

Neurobehavioral Examination

Attention and Memory: Attention span was within normal limits. Initial registration of items to be remembered was mildly impaired. Immediate sentence recall was low average. However, recall after a 5-minute delay was severely impaired. The patient did not perseverate, but did fill gaps in her recall with unrelated information.

Language: Speech was fluent without significant word-finding problems or paraphasic errors. Lingual, labial and guttural sounds were intact, but a mild central dysarthria was noted. Rhythm, prosody, and phonation were within normal limits. Naming to confrontation was intact. Comprehension was intact for three-step commands. She was able to read single letters, and numbers (Block Print), but was not able to see well enough to read sentences. Her ability to perform mental arithmetic calculations was well above average.

Motor Programming and Praxis: Fine motor control was mildly impaired on the left hand, but within normal limits on the right. Alternating hand movements were also mildly impaired. Inhibitory paratonia (Gegenhalten) was observed on the right. Facilitatory paratonia (Modified Kraul Procedure, grade 4) was noted on the left. Mild motor disinhibition was also noted. However, the patient was immediately aware of these errors, and self-corrected them. There was no sign of ideomotor or buccofacial apraxia.

Visual Spatial and Constructive: Somatosensory function was intact to double simultaneous stimulation. Finger localization, graphesthesia and stereognosis were intact bilaterally. Copy of drawings was impaired due to deficits in visual acuity. However, spontaneous drawing (Draw-a-Clock) was well preserved.

Executive Functions: Verbal abstract reasoning proverb interpretation, judgment and insight were intact. Copy of Alternating Figures and Letters was confounded by visual difficulties.

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