4.
Lateral Medullary (Wallenberg) Syndrome
5.
Acoustic Neuroma
6.
Weber Syndrome (Rostral Alternating Hemiplegia)
7.
Cerebellar Cases (2)
8.
Broca’s Aphasia
9.
Huntington’s Disease
10.
Cataract
11.
Several Hearing Loss Cases
12.
Thalamic Lesion-Pure Sensory Loss
13.
Subdural Hematoma
14.
“David and Goliath” (my favorite!!)
When
the 9 scenarios below are added to the 14 above, you will have 23 neuro
vignettes in your long-term memory (your “blackboard” can’t hold them all
long enough!).
15.Clinical
Vignette--ALZHEIMER’S
A
52 year-old man is noted to have memory difficulties.He
forgets to pay several bills and forgets numerous business appointments.His
wife reports that he appears depressed and is not his usually jovial self.He
acts strangely and inappropriately and has an affair with a 15 year-old
high school sophomore.Neurological
examination shows the following abnormalities.
1.
disorientation for year, month and day
2.
inability to remember 3 unrelated objects at 15 minutes
3.
inability to accurately draw geometric shapes
4.
inability to think abstractly.
CT and MRI show no evidence of brain atrophy; MRI-SPECT
(spectroscopy) shows reduced metabolism/blood flow in frontal, temporal
and parietal regions bilaterally.
JKH
Comment:
This
patient has dementia, most likely of the pre-senile Alzheimer type.In
the early stages of this disorder, there may be clinical evidence of cognitive
or intellectual deterioration; however, CT and MRI may not yet show evidence
of brain atrophy (shrinkage).In
the later stages, there should be anatomical evidence of brain atrophy
and pathological studies would show loss of neurons.There
are other pathological changes e.g. neurofibrillary tangles, amyloid deposits.MRI-SPECT
shows physiological evidence of reduced perfusion and presumed reduced
metabolism.This is more sensitive
test than CT/MRI.Although there
is reduced brain perfusion, techniques to increase cerebral blood flow
will not enhance brain function.The
reduced brain perfusion is a manifestation of primary neural depression;
therefore as there is reduced brain mass, there is need for reduced blood
flow.In Alzheimer dementia, there
is a central cholinergic disorder.Drugs
that enhance acetylcholine function are effective in enhancing memory (physostimine
is an inhibitor of the catabolic enzyme of ACh and therefore increases
the level of ACh; carbachol=cholinergic agonist).
16.Clinical
Vignette--PARKINSON’S
Louise
Bermel is a 60 year old female who presents to the office with difficulty
using her right arm.Ms. Bermel states
that the difficulty in using her right arm has occurred over the past 2
months.She is right-handed.The
clumsiness and intermittent tremor have become progressively worse.No
other symptoms are present.To direct
questioning she says the right shoulder aches, "it may be bursitis," and
that she has noted saliva on her pillow in the mornings.Her
children feel she is slower and looks depressed.She
denies any visual disturbances, vertigo, palpitations, sensory symptoms
or unconsciousness.She denies headaches
and there is no history of trauma.
The
problems with her shoulder and arm are making it more difficult for her
to carry out her work.There is no
history of neurological diseases in the family except for her mother who
had a slight shake of her head in old age.There
is no history of early cerebrovascular or coronary artery disease in the
family.There is no family history
of hypertension.Her father died
of pneumonia and her mother died of cancer of the breast.
Neurologic
Exam:Normal cranial nerves II-XII,
finger to finger, heel to shin, on gait testing her right arm swings less
and on turns her neck looks stiff.Deep
tendon reflexes, sensation and fine coordination are almost normal.There
is no significant weakness.Initiation
of gait is slow and a little unsteady but when pulled from behind she does
not lose her balance.There is a
slight tremor of her right hand intermittently at rest that disappears
when she reaches for something.It
is also noted when she is walking.
JKH
Comment:
This
is a case of Parkinson’s that often begins unilaterally.An
MRI could be done, but the presence of a tremor would interfere with getting
a good exam.Treatment is designed
to maintain normal function--the lifestyle to which she was accustomed--which
has tremendous psychological benefit for her.The
surgical possibilities are pallidotomy, deep brain stimulation and fetal
transplantation.
17.Clinical
Vignette LOW BACK PAIN
A
45 year old delivery man comes to see you complaining of low back pain
that has been intermittent for the past 6 months.The
pain is in the middle of the lower back and usually radiates into the left
buttock.The pain is made worse by
sneezing, coughing, or when he hits a pot hole while driving.In
some cases these maneuvers cause the pain to radiate down the back of his
left leg into the bottom lateral aspect of his foot.Over
the past 6 weeks, he has noted that it is difficult for him to stand on
his tip toes and that this is primarily because of weakness in his left
foot.
On
exam he has a normal neurological exam except that his left ankle jerk
is absent and he has weakness of his left gastrocnemius.There
is abnormal sensation over the lateral aspect of the left foot.He
cannot stand on his toes of his left foot.When
you have him lying down, you cannot elevate his left leg above 35 degrees
because of shooting pain into his left buttock and down the back of his
left leg.
18.Clinical
Vignette-Amyotropic Lateral Sclerosis
A
65 year old man presents with a six month history of progressive fatigue,
weakness and leg cramps.On a few
occasions he choked on food.His
wife noted diffuse twitching of muscles on his chest and upper back. Two
months ago he developed a foot drop in his left leg.He
has not complained of any sensory symptoms.There
has been no cognitive decline. He has no difficulty with bowel or bladder
function.His family history is
noncontributory.
Examination
showed that the patient had normal mental status.Motor
examination showed severe, bilateral diffuse muscle wasting in both upper
and lower extremities. The most atrophied were the deltoid, triceps, biceps,
hand muscles and quadriceps on either side and the left anterior tibialis.There
were prominent fasciculations in all muscle groups.The
muscle tone was increased in both upper and lower extremities.There
was diffuse weakness in all 4 extremities with complete left foot drop.Neck
extensors were profoundly weak so that the patient was barely able to keep
his head up.The tendon reflexes
were hyperactive in all four extremities. The plantar reflexes were extensor
(Babinski sign).Sensory examination
and coordination were normal.His
gait was characterized by decreased arm swing and limping of the left leg.
19.Clinical
Vignette--Syringomyelia
A
56-year old woman has a long history of numbness in and clumsiness of her
hands.Initially the numbness started
in her right hand but progressed slowly to include both hands and both
arms.Over the past year the numbness
has actually included the shoulders, neck, scalp and most recently has
included the area of the face next to the hairline and the angle of the
jaw on the right.Besides the numbness,
she has noted increasing difficulty in opening jars and over the past 2-3
years has had trouble brushing her hair.Most
recently she has begun to have trouble with slurred speech, swallowing,
and a hoarse voice.
Her
other complaints include trouble with walking.It
seems that she is having trouble with tripping over her toes.She
also states that she urinates 10-15 times per day and that she needs to
get to the rest room quickly otherwise she can loose her urine.
She
has smoked cigarettes for many years and recently stopped because she has
burned her fingers a number of times and did not feel the pain.She
had to have a skin graft of her right hand because of a severe burn she
sustained from a stove.
Her
exam revealed a normal mental status.Cranial
nerve exam showed a bilateral decrease in pin prick sensation at the hair
line and angle of the jaw.The palate
moved poorly and her voice had a nasal characteristic.Her
gag reflex was depressed.There was
some atrophy of the left side of her tongue with fasciculations noted.The
motor exam revealed diffuse weakness and atrophy of the upper extremities.There
were some fasciculations noted in the biceps bilaterally.The
reflexes in the upper extremities were barely obtained.In
the lower extremities there was mild weakness and increased tone and reflexes.Bilateral
Babinski signs were noted.The patient’s
gait was stiff and she could not walk on her heels.The
sensory exam in the lower extremities was normal.The
sensation in the upper extremities was intact to vibration and joint position
sense but she could not feel pin prick in either upper extremity or across
the shoulders, the neck or the scalp.
20.Clinical
Vignette--Wernicke-Korsakoff Syndrome
A 50 year-old CEO develops abdominal pain and is found to have a tender abdomen on examination.Liver function studies are markedly abnormal and serum amylase is markedly elevated.An abdominal CT shows a mass.At surgery, he is found to have a pancreatic pseudocyst.(The pseudocyst is, by far, the most common cystic mass of the pancreas.) Approximately 10% of patients develop pseudocysts following several episodes of alcohol induced pancreatitis (severe complications of pancreatitis are more common when alcohol induced). These cysts are usually noncomplicated. If complicated, and there is no history of pancreatitis, the diagnosis of mucinous cystic neoplasm must be entertained. This may seem to be an obvious distinction, but many mucinous cystic neoplasms carry a diagnosis of pseudocyst before the correct diagnosis is made.In the surgical recovery room, he awakens and is delirious and tremulous.He is treated with CNS tranquilizers and this calms him.Two days later, he awakens but his recent memory is quite poor but remote (past) memory is intact.He can not remember any of three unrelated objects at 10 minutes.
JKH
Comment:
Based
upon the clinical features, this patient most likely has alcohol-induced
damage even though no statement is made to suggest "chronic alcoholism."Initially,
he is "delirious" and "tremulous" and this suggest major alcoholic withdrawal
syndrome which occurs after abrupt cessation of alcohol.As
the patient awakens and shows poor recent memory (like H.M.), this is consistent
with Wernicke-KorsakoffSyndrome.One
way of telling this disease from anoxia of the hippocampi (both exhibit
the absence of new memories) is that with Korsakoff’s there is “confabulation,”
that is, the patient tries to cover his memory loss by making up
stories.
Pathological
changes include necrotic and hemorrhagic microscopic lesions involving
the mammillary bodies (memory problem due to connection with hippocampus
via fornix) periaqueductal midbrain, paraventricular thalamus and hypothalamus.This
disorder results from specific deficiency of vitamin B-one (thiamine).This
is an essential cofactor for decarboxylation of pyruvate and alpha-ketoglutarate.Replacement
therapy with thiamine should cause improvement in memory function.
21.Clinical
Vignette-Multiple Sclerosis
Leslie
Biggins, a twenty-eight year old, male, graduate student presents complaining
of double vision when he looks to the right for the past three days.Mr.
Biggins states that the double vision was first noticed upon awakening
three days ago.He feels fine and
has no other complaints.He has
never been ill with the exception of an occasional upper respiratory infection
and the usual childhood diseases of measles, chickenpox and mumps.His
family history is negative except for hypertension in his father, which
is well controlled with medication.His
maternal grandmother was afflicted with "rheumatism" and was in a wheel
chair for many years before her death.
He
reports no headaches, muscle weakness, dizziness or sensory anomalies.He
sleeps poorly and is up most of the night because of need to urinate frequently.He
feels that this is a direct consequence of caffeine intake.Two
months ago he became incontinent of urine in bed, and he has cut off coffee
altogether.He feels that this has
improved matters somewhat, but not entirely.He
has noticed an inability to concentrate and fatigue has become an inordinate
problem.
He
is not currently taking any medication and does not smoke or drink.He
is a graduate student in clinical psychology, under much pressure to finish
his dissertation.His wife is anxious
to start a family.He admits to
being "stressed out" and depressed.This
has had a significant negative impact regarding his relationship with his
wife and recently he has had problems with impotence.
Neurological Examination: (I have noted only important ones!)
Mental
status:Mr. Biggins is cooperative
and is oriented as to place and time.He
has appropriate memory for immediate (working), recent (hippocampal) and
past events.He is able to compute
arithmetically and can attend to similarities and differences.
Eyes:On
right gaze the right eye abducts but the left eye cannot pass the midline.Nystagmus
is present in the right (good) eye during right lateral gaze.On
left gaze both eyes turn left normally without nystagmus or diplopia.
Motor
System--Upper and lower limbs were strong.
Tone
in both lower extremities is elevated.Clonus
was elicited in the left ankle.
Sensation:Proprioception,
vibration, pain, and temperature were all normal, as was stereognosis and
two-point-discrimination.
DTR: Symmetrical and 3+.
Babinski
+ Bilaterally.
Cerebellar
funct.:Gait, toe-heel, finger-nose,
rapid alternating movement are normal and symmetric.
The
MRI shows multiple high signal (white) white-matter lesions.The
lesions are mostly around the lateral ventricles.A
number of the lesions are oval in shape and some lesions also appear as
dark "holes" in the T1 images.
22.Clinical
Vignette--EPILEPSY
A
two-year-old boy presents to the ER.Just
after awakening, the mother noticed that his right arm, then leg began
to shake.He was also "out of it"
but not unconscious; he would not respond to her.This
continued while she brought the child to the ER.He
had been fine that day except for feeling "warm."On
arrival to the ER, the right-sided rhythmic jerking movements continues.The
rest of the exam was significant for a temperature of 1040and
a left otitis media.Past medical
history was significant for speech delay, as he did not yet have any words
beside "mama" and "dada."
JKH
Comment:
The
two-year-old boy presented above has a simple partial seizure
involving predominantly the left hemisphere (right sided jerking movements).The
MRI showed a subependymal heterotopia on the left.Gray
matter heterotopias are collections of normal neurons in an unusual location,
due to an arrest in fetal neuronal migration.Because
of its localization, these neurons have escaped the cortical GABA-ergic
inhibition that normally prevents over excitation.Because
of this lack of inhibition, these misplaced neurons are highly epileptogenic.Increased
body temperature, such as one sees during a fever, increases the excitability
and can provoke a seizure.
23.Clinical
Vignette—Myasthenia Gravis
A
25 year old woman came to your office complaining of intermittent double
vision for the last three weeks.She
also has complained of fatigue.She
has felt best during the early morning hours, but later, during the course
of the day, she gradually develops double vision and diffuse weakness.Her
boy-friend has observed that her right eyelid has been drooping frequently.She
used to play competitive basketball while in college, but now she has been
short of breath after climbing only 2 flights of stairs.Also,
on a few occasions, she choked on food and her friends noted that her speech
was slurred or thick.
On
examination her mental status was normal.She
had marked ptosis on the right side.Eye
movement examination showed decreased movement in all directions in the
right eye.There was also slightly
decreased abduction in the left eye.On
repetitive blinking she developed ptosis of the left eye lid and her right
sided ptosis got much worse.Motor
examination showed normal muscle bulk and tone.Muscle
testing revealed that she was initially strong, but rapidly became “tired”
or weak with repeated effort.She
was unable to hold her arms abducted at 90 degrees for more than 30 seconds.All
sensory modalities, reflexes, coordination and gait examination were normal.Plantar
reflexes were flexor.
JKH Comment:
Myasthenia
gravis is an immune mediated disorder of neuromuscular transmission.This
condition is characterized by fluctuating weakness and fatigability of
voluntary muscles.In the vast majority
of cases, extraocular muscles are involved.Some
patients may also have involvement of the muscles of the tongue, pharynx,
and soft palate (dysphagia; difficulty swallowing and dysarthria; slurred
speech).Weakness gets worse with
any sustained activity and improves after a period of rest.
MG
is caused by immune mediated destruction of acetylcholine receptors at
the postsynaptic membrane and in some patients it may be associated with
a thymoma (tumor) of the thyroid gland.(About
one in ten MG patients has a thymoma).