Case 1: Vertigo
Chief Complaint: A 58-year old diabetic man developed nausea and dizziness.
History of present illness: On the day of admission, a 58-year old left-handed pastry chef awoke with a dull headache, nausea, and a sense of spinning. When he attempted to stand, he felt himself tending to fall to the right, although did not lose balance. He initially attributed the symptoms to a migraine headache, triggered by a glass of red wine before bed. As the morning progressed, his dizziness increased, and he noted slurred speech and clumsiness with the right hand. A friend drove him to the Emergency Room.
Review of systems: There was no history of vomiting, diplopia, blurred vision, hearing loss, weakness, or numbness. There was no recent history of head or neck trauma, chest pain, palpitations, dyspnea, or other symptoms.
Past medical history:
1. Type II diabetes of 5 years’ duration, treated using glipizide [Glucotrol]
2. Hypertension of ten years’ duration, treated using enalapril [Vasotec]
3. Hypercholesterolemia, treated using atorvastatin [Lipitor]
4. Occasional headaches
Family history: His mother was alive at age 88, but had hypertension, diabetes and dementia.
His father had died at age 66, following a myocardial infarction. His only sibling, a brother, underwent a triple CABG at age 50. His brother suffered from migraine headaches.
Personal history: He was a former 1 pack per day smoker for 15 years, having quit at age 31.
He drank up to two glasses of wine most evenings, and had a fondness for single malt whiskey.
Examination: The patient was a heavy-set 58 year old man who looked anxious. His blood pressure was 190/100 and his heart rate was 76 and regular. His weight was estimated at 230 pounds. He was alert with normal cognition and language function, but his speech was slightly dysarthric, with an irregular cadence. Visual fields were full and the fundoscopic exam was normal. His right pupil was small and reactive, with a slightly drooped upper eyelid. He had right-directed horizontal nystagmus when looking to the right, accompanied by a rotatory component. He had decreased hearing in the right ear. His facial sensation was decreased over the right side, and there was absent perception of pinprick and temperature over the left body. Bulk, tone and power were normal in all muscles. He had
impaired coordination in the right hand, with dysmetria and past-pointing in a target-following test.
His right coordination was impaired on the heel-to-shin maneuver. He was unable to stand, tending to topple to the right.
Course in hospital:
Within one hour after arriving in the ER, he suddenly complained of being unable to see. He then became unable to speak or move, and seemed unable to move his eyes. His blood pressure was
197 210/120 and the heart rate 60. He breathing became shallow and irregular, and he made gurgling noises. He was intubated, and placed on a ventilator. On neurological examination he was
unresponsive. His pupils were small, but remained slightly reactive to light. The fundoscopic exam was difficult to perform due to pupillary miosis. Using oculocephalic maneuvers, his eyes could not be deviated horizontally or vertically. His corneal reflexes produced no response. His motor exam
showed a flaccid paralysis of all limbs, with no withdrawal responses or localization to pain. His deep tendon reflexes were hypoactive, and the plantar responses were indifferent.
The patient underwent a cranial imaging study before transfer to the NICU. On arrival to the NICU, approximately 12 hours after his initial symptoms, he was unresponsive, apneic, with no brainstem reflexes. His pupils were 6 mm, dilated and unreactive.
Issues to discuss:
1. What is the localization of this patient’s initial exam findings? What neurological structures are involved in his disease process? In which vascular territory are the patient’s initial symptoms and signs?
2. What are the hallmarks of brainstem localization? What is a Horner syndrome?
3. What happened to this patient after arrival in the ER? What additional neurological structures were affected by his deterioration?
4. What are the clinical criteria for brain death?
5. What were this patient’s risk factors for cerebrovascular disease?
Case 2: Forgetfulness
Chief complaint: The patient was an 81-year old man with slowly progressive memory loss.
History of present illness: The patient was a retired electrical engineer who worked until the age of 70. In the years following his retirement, the patient gradually became forgetful, often losing objects or missing appointments. He became slower at decision-making, and had difficulties with recall of small details. Formerly a fastidious hobbyist, the patient could no longer assemble model airplanes, which now made him frustrated and irritable. Gradually, he became unable to manage the household finances, complete a shopping list, or set the table for dinner. He tended to wander around his
neighborhood without knowing how to find his way home. On one occasion, he got into the subway, and was brought back by the police. When he was subsequently confined to his house, he insisted that people were plotting to kidnap him. His sleep was disturbed with frequent awakenings during which
he would call out to his wife for help. During the day, he slept for 3 hours, and often sat in his chair looking out of the window.
Review of systems: There was no history of systemic illness, seizures, myoclonus, headaches, visual deficits, hearing loss, weakness, tremor, dysarthria, or bladder impairment. He did not have
hypertension, diabetes, tobacco use, toxin exposure or excessive alcohol intake.
Family history: Negative for dementia or other neurological disorders. A son suffered from schizophrenia.
Examination: On examination, he was slightly disheveled, but pleasant, alert and cooperative. He did not seem to know why he was being examined, and could not give the date or location. He tended to cover up his errors, stating, “Why do you ask me these silly questions?” His speech was fluent and articulate, but he often lost track of his train of thought. Sometimes, his speech was laden with technical terms from his engineering background, but did not make sense. His repetition was intact.
When asked to write a sentence, he wrote his first name. He could not copy a cube. He could read aloud, but not follow a written command, or perform a two-step command. He had difficulty imprinting 3 objects, and could remember none subsequently. When shown a standard series of pictures, he named a wristwatch a “clock”, and a dolphin a “pluke”. When asked to list the past five
Presidents, he could name only Kennedy and “the peanut farmer.” He could not subtract serial 3s from 20. The digit span was 4 forwards, but he could not repeat the sequence backwards. He tended to perseverate on performing tasks of the neurological exam. When asked to pantomime simple actions,
he performed adequately.
The cranial nerve exam was normal. The motor exam showed active resistance to passive movement, but no weakness or spasticity. Bilateral grasp reflexes, palmomental reflexes, and a snout reflex were
elicited. Sensory exam, reflexes, coordination and gait were all completely normal.
Laboratory:
Neuropsychological testing - The patient had undergone neuropsychological testing at age 76 and 81.
Over this interval, the full scale IQ declined from 125 to 99. Prominent deterioration was seen in short-term recall, verbal memory, language skills, spatial perception and executive function.
199 Electroencephalogram [EEG] – Mild, diffuse, nonfocal background disorganization and slowing.
Cranial MRI - Mild cortical and subcortical atrophy.
Questions for discussion:
1. How is a diagnosis of dementia made?
2. Differentiate between dementia, delirium and pseudodementia.
3. What cognitive deficits are revealed by the neurological exam, and to what parts of the cerebrum do they localize?
4. What is dysphasia? What is a paraphasic error?
5. List some causes of dementia, and describe how you would diagnose and treat them.
4. What is the most likely cause of dementia in this individual?