Neurology Clerkship Information/Study Guide
Neurology Clerkship Information/Study Guide
WELCOME
TO
NEUROLOGY
I swear by Apollo Physician, by Asclepius, by Health, by Panacea, and by all the gods and
goddesses, making them my witnesses, that I will carry out, according to my ability and
judgment, this oath and this indenture .... I will use treatment to help the sick according to my
ability and judgment, but never with a view to injury and wrongdoing. I will keep pure and holy
both my life and my art. In whatsoever houses I enter, I will enter to help the sick, and I will
abstain from all intentional wrongdoing and harm .... Now if I carry out this oath, and break it
not, may I gain forever reputation among all men for my life and for my art-, but if I transgress it
and forswear myself, may the opposite befall me.
Oath of Hippocrates
NEUROLOGY TEXTS
:
*Principles of Neurology. Companion Handbook. R.D. Adams and M. Victor, 4th Edition,
McGraw Hill Book Company.
*Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology (3rd Edition). Little, Brown
and Company, 1996.
INTRODUCTION
Unless otherwise specified, "Chairman's Rounds', are held once weekly on each service. In spite
of the whispered stories you may have heard, it is not a punishment but rather a teaching exercise.
Remember this is not a graded exercise. Your grade is determined by your attending physician
and your test, not by chairman's rounds. Nevertheless, we recognize that presentations in front of
groups can be disconcerting, and some nervousness is natural.
THE FORMAT
The presentation will be a complete history and neurologic examination of a patient. The
chairman is at his liberty to interrupt and ask you to elaborate upon a historical issue or clinical
finding. To ensure the rounds go smoothly one should have the pertinent radiological folders
handy (CT, MRI, angiogram ... ), but do not give lab tests or radiologic tests as part of the history
or objective findings. This is a clinical exercise. The studies will be looked at the end and see
how your clinical diagnosis correlates with the paraclinical investigations.
Appendix 2
APHASIAS
SENSORY MOTOR MIXED
BROCA WERNICKE CONDUCTION GLOBAL TRANS- TRANS- TRANS-
CORTICAL CORTICAL CORTICAL
NON- NON- NON- NON-
SPONTANEOUS SPEECH FLUENT FLUENT FLUENT
FLUENT FLUENT FLUENT FLUENT
AUDITORY GOOD- SEVERELY GOOD- VERY
GOOD POOR POOR
COMPREHENSION NORMAL IMPAIRED NORMAL POOR
READING GOOD-
COMPREHENSION
GOOD POOR
NORMAL ? POOR GOOD POOR
VERY
NAMING POOR POOR POOR POOR POOR POOR
POOR
GOOD- GOOD-
REPETITION POOR POOR POOR IMPAIRED GOOD
NORMAL NORMAL
PARAPHASIA - + + ? + - +
Form of Examination
History
1. Please record the name and relationship of the person from whom the history is obtained if
other than the patient.
2. Record verbatim the patient's chief complaint. If there are many, ask them to tell you what their
chief problem is.
3. Pursue individual symptoms as if you were a reporter, i.e., where, what, why, when, details
are important, For example, if the patient is having episodes of confusion, ask about the first
spell, how long it lasted, how it evolved (fast onset, gradual, fast defervescence, etc.), associated
symptoms, time of day, what was being done at the time, how like all other spells was the first,
etc. 80-90% of the diagnosis is history and you are likely to be out of luck if you don't know
where you are going by the end of the history.
4. Next, past medical history and surgical history, habits, medications, family history, and review
of systems.
Physical Symptoms
a. This includes rectal on all patients, breast exam, genital exams - pelvic examination for
women and testicular examination for men.
b. Please remember that the eyes and ears are sensory organs. Record the examination of
the auricle, external auditory meatus, TM, cornea and conjunctiva, in the general exam
and save Rinne, Weber, visual acuity, visual fields, fundus, pupils and ocular motility for
the neurologic exam.
c. In the general examination, examine the skull, neck and back, paying particular
attention to structural defects, bruits and painful sites
Mental status
Cranial nerves
Motor system strength (proximal versus distal - major groups)
tone
reflexes
pathologic reflexes
Cerebellar
Sensory system
Station and gait
Present your written record of the patient contact in the same compulsively organized fashion
with which you obtained the information. Remember, if you stray from your organization, you
will forget important items and the record will be a morass to the next reader.
2. Formulation
At the end of the history and physical examination, formulate the clinical problem in a capsule
paragraph which summarized the pertinent findings.
3. Diagnostic impression
4. Differential diagnosis
Please read about the disease you have diagnosed and be prepared to give basic information about
the disease process and the differential diagnosis.
5. Plan
Discuss how you will evaluate each of the unknowns in your differential diagnosis and how you
will treat each of your knowns.
6. Caution
Whenever you write a note in a patient's chart, you are contributing to a legal document. Under
no circumstances should such an entry contain argumentative issues or demeaning statements
about the patient or other members of the health care team. Therefore, make only those
statements behind which you stand and on which you are going to act. For instance, if you do not
intend to study the patient in any way to prove or disprove that he has a remotely possible
diagnosis, don't say "rule out" that diagnosis in the chart.
2. You should know the major groups of neurologic diseases and be familiar with the texts to
which you can turn for more information.
3. You should be able to formulate a neurologic problem in terms of the basic anatomy and the
basic kinds of disease which affect that part of the anatomy.
4. You should have a good direct or indirect exposure to a variety of neurologic disease.
Expectations
Some students are upset at the number of physical examinations they are required to do, the
length of rounds, and the amount of "scut" work there is to do. You are an integral part of a busy
service. Without your assistance and insights, everything runs less efficiently and is done less
well. This is not unique to neurology, nor to this school, nor to medicine. There is no simple way
for us to do pre-digest and deposit neurology as a discipline into the minds of students. We will
be happy to give you readings to do about your patients. There will be many mini-lectures and
discussions during rounds. You will be expected to participate by knowing-.
1. how your patients are every day
2. what tests they are to be having
3. how the examination has changed from admission
4. at least the bare minimum of reading on each case.
If you expect to be given honors on the rotation, you must exceed those minimum requirements
and demonstrate that you are doing more than the bare minimum. The excuse that there is "no
time to study" is unacceptable.
Presentations
You will be presenting your patients to the assembled groups. Be prepared to present the history
and physical concisely, covering the pertinent negatives as well as the positive findings. For
example, when giving a history on a patient with episodic loss of consciousness, ask about "deja-
vu", "jamais-vu", disorientation, unusual smells and tastes. Report these as present or absent
because they are germane to this symptoms.
Please do not continually say "the patient denies" since this connotes disbelief or incredulity.
Simply state what the patient's response was - e.g., "The patient does not smoke and drinks
socially." If you do not believe the patient, you should have evidence such as "but has cigarette-
stained hands" or "but smelled of alcohol."
2. Formal Lectures
The faculty believes that the didactic lectures in Neurology are important. These will be given
daily during your rotation. The lectures are a supplement to your independent study and your
ward experience. The lectures also give you the opportunity to ask questions. We expect your
attendance at these lectures. We do not consider the availability of "canned lecture notes" as an
alternative to attendance to the lectures.
3. Independent Study
After much discussion and review of several neurological texts, the faculty recommends:
2. Principles of Neurology, Companion Handbook. R.D. Adams and M. Victor, 4th Edition,
McGraw Hill Book Company.
Evaluation by Faculty/Residents
Your performance on your clerkship will be evaluated by the faculty and residents. They will
evaluate your ability to obtain a neurologic history, to perform a neurologic examination, to
formulate a diagnosis and to devise a plan for further investigation.
Your evaluation will also include the faculty and resident's assessments of your fund of
knowledge in Neurology. Your rapport with patients, colleagues and other medical personnel will
also be judged.
Age ____________
"Most headaches seen by physicians are vascular (migraine, cluster), scalp muscle contraction
(tension), or combined vascular and scalp muscle contraction headache."
- Onset
· Location-
Intensity-
Duration-
Pattern (i.e.: continuous, periodic, etc.)-
Associated symptoms (i.e.: photophobia, sonophobia, nausea, vomiting. visual complaints)
Amaurosis Fugax
Diplopia
- Deafness -
Tinnitus
V. Deglutition/Phonation
- Dysphagia -
Dysphonia
VI. Speech-
Dysarthria -
Aphasia (see classification of aphasias; Appendix 2)
Syncope-
Seizure-
Coma
- Insomnia-
Excessive daytime sleepiness
- Narcoleptic Syndrome-
Sleep Apnea Syndrome
X. Sensory Complaints
- Incontinence-
Urinary retention-
Constipation
- Impotence-
Impaired erection-
Impaired ejaculation
B. PAST HISTORY
Hypertension
Cardiac Disease - ischemic
Cardiac Disease - valvular
Cardiac Arrhythmia
Diabetes
Hyperlipidemia
Cigarette smoking
Alcohol consumption
Migraine
Oral contraceptives
Exposure to neurotoxins
Drug abuse
Sexual behavior
Head trauma
Previous seizures
Childhood illness
Syphilis
Obstetric trauma
Perinatal history
Hypertension
Cardiac disease
Diabetes
Stroke
Stroke - under age 50
Neurological/Mental disorders
D. NEUROLOGICAL EXAMINATION
a. Level of Alertness
b. Orientation
- person-
place-
time
C. Attention
d. Memory Tests
- recent events-
three names-
number sets
2. Symbolic Functions
a. See language
b. Praxis
c. Gnosis
3. Skull
a. Head circumference
b. Palpation
c. Auscultation
4. Neck
a. Meningeal Signs
- neck stiffness
- Kernig
- Brudzinski
b. Range of Motion
c. Bruits (Grade I-VI)
6. Upper Limbs
a. Bulk
b. Tone
C. Strength
0. no trace of movement
1. flicker of muscle contraction C5 (Sh Abd, Elb Flx)
2. some movement around a joint C6 (Wr Ext)
3. movement against gravity C7 (Elb Ext, Wr Flx. Fing, Flx)
4. movement against some resistance C8 (Fing Flx.- intrinsics)
5. full strength TI (Fing Abd, Fing Add)
d. Coordination
Finger-Finger
Finger-Nose
Rapid alternating movements Check-response (Stewart-Holmes)
e. Sensory (Appendix 4)
Pain
Light Touch
Temperature
Position
Vibration (128 Hz tuning fork)
Cortical Sensory Modalities
stereognosis
graphesthesia
two-point discrimination
f. Reflexes
0. Absent
1. Hypoactive
2. Normal
3. Increased
4. Increased often with clonus
1. Biceps (C5-C6)
2. Brachioradialis (C5-C6)
3. Triceps (C7-8)
4. Finger Flexion (C8-TI)
7. Trunk
c. Sensory (Appendix 4)
Pain
Light touch
Temperature
Vibration
d. Spine
Inspection
Palpation
Auscultation
8. Lower Extremities
a. Bulk L3 (Knee Ext)
b. Tone L4 (Ank- dorsiflex, inversion)
c. Strength L5 (EHL, Toe Ext)
d. Coordination SI (Ft Ev, Ft Plnt Flx, Glt Max)
Heel-Knee-Shin S2 (Ft intrinsics)
e. Sensory (Appendix 4)
Pain
Light touch
Temperature
Position
Vibration (I 28 Hz tuning fork)
Cortical sensory modalities (see 6e)
f. Reflexes
Cremasteric (LI -L2)
Patellar (L2-L4)
Achilles (SI-S2)
Plantar Response
9. Gait-Station
Arm Swing
Width of Gait
? Limp
Toe Walking
Heel Walking
Tandem Walking
Romberg Test
10. Summary
a. Anatomical Diagnosis (Where is the problem?)
c. Pathogenesis (Why?)
d. Evaluation
I What is the year, season, date, day of the week, month? (5 points)
2. Where are we: country, state, city or town, hospital, floor of building? (5 points)
3. The examiner names three objects, taking 1 second to say each. The patient is asked to repeat
the names of all three objects after the examiner has said them. (3 points)
4. Serial sevens. One point for each correct answer. Stop after five answers. Or spell the word
"world" backwards. (5 points)
6. The patient is shown a pencil and a watch and asked to name them. (2 points)
8. The patient is handed a sheet of paper and asked to carry out a three-stage command: "Take
this paper in your night hand, fold it in half, and put it on the floor." (3 points)
9. The patient is shown a sign that reads "Close your eyes" and is asked to obey the sign. (1 point)
Interpretation of Results:
José Biller, MD
Indiana University School of Medicine
Indianapolis, Indiana
Arterial Hypertension 6x
Cardiac Disease 2-6x*
Transient Ischemic Attacks
(TIA's)10x
Prior Strokes 10x
Asymptomatic Carotid Bruits 3x
Diabetes Mellitus 2-4x
Cigarette Smoking 2x *Rheumatic Afib, relative
risk=17x
Transient isolated vertigo, diplopia or dysphagia are not specific for the diagnosis of
vertebrobasilar TIA.
Thromboembolism Non-Atherosclerotic
Cardioembolism Vasculopathies
Impairment of Distal Flow Small Vessel Disease (Lacunes)
Hematological/Rheological Mechanical Interference with
Disorders Blood Flow
Steal Syndromes
The use of anticoagulants has also been recommended by some authorities for patients who
sustain recurrent episodes of focal cerebral ischemia despite platelet antiaggregants.
However, recent studies comparing prophylactic oral anticoagulants with platelet
antiaggregants, suggest that although oral anticoagulants reduce the risk of stroke in
patients with threatened stroke, neither study showed a significant difference between the
two groups.
Conclusions:
Ischemic stroke is a major cause of death and disability. Despite its high incidence, acute
management remains controversial. Most current forms of therapy are designed to reduce
complications of a recent stroke or prevent recurrences. Experimental data suggest that the
optimal time for intervention should be the immediate hours following brain ischemia.
f. Brain Tumors
1. Primary
a) malignant
b) benign
2. Metastatic
a) choriocarcinoma
b) melanoma
c) lung
d) renal
C. CLINICAL PRESENTATION:
P General manifestations
P Manifestations according to location
Putamen
Lobar
Thalamus
Pons
Cerebellar
Others
D. EVALUATION:
History
Physical examination
Neurological examination
Laboratory screen
CBC, Platelet, PT, PTT, ESR
Blood chemistries, electrolytes
Urinalysis
CT of brain
Chest roentgenogram
12 lead ECG
In Selected Cases:
E. MANAGEMENT:
Airway adequacy
Raised intracranial pressure Rx
ICP monitoring (?)
Blood pressure Rx
Anticonvulsants (?)
Surgery
Early diagnosis
CT
LP if CT is negative
Cerebral angiography (4 vessel)
Clinical grading
Vasospasm Rx
Prevention of rebleeding
SEIZURES
A. DEFINITION - SEIZURES:
Transient disturbance of cerebral function due to abnormal neuronal discharges.
u Epileptic seizures
u Non-epileptic seizure
(Syncope, metabolic derangements, parasomnias, movement disorders)
Psychogenic
B. DEFINITION - EPILEPSY:
Disorders characterized by a predisposition to recurrent seizures.
Active cases on therapy: 45-100 million worldwide; 1.5-3.5 million in USA.
Absence
Tonic-clonic
Clonic-tonic-clonic
Clonic
Myoclonic
Atonic (drop attacks)
Tonic
simple absence
clonic components (myoclonic absences)
increased tone (retropulsive absences)
decreased tone (atonic absences)
automatism (automatic absences)
autonomic phenomena
little or no warning
EEG definitely abnormal at start
loss of posture with high risk of self injury
loss of consciousness
bladder or bowel incontinence likely
violent contraction of limb and trunk muscles
post-ictal confusion lasting minutes to hours
Convulsive
tonic-clonic status
myoclonic status
clonic-tonic-clonic status
Non-convulsive
idiopathic
symptomatic
trauma
congenital malformations
genetic disease
infections (e.g. meningitis, encephalitis)
metabolic derangements (e.g. hypoglycemia, hypocalcemia, hypo/hypernatremia)
drug overdose (e.g. tricyclics, theophylline, lidocaine)
drug withdrawal (e.g. ethanol, sedative-hypnotics)
tumors
cerebrovascular disorders
Q. EVALUATION:
history
general physical examination
neurological examination
EEG
blood screen (glucose, BUN, creatinine, lytes, CBC, ESR, LFTs, ABGs, drug
screen)
CT or MRI
If CNS infection suspected - CSF
DEMENTIA
B. EPIDEMIOLOGY:
4-5% of persons over age 65 have severe dementia
10% of persons over age 65 have mild to moderate dementia
First most common type - Alzheimer's Disease
Second most common type - Vascular Dementia
C. CLASSIFICATION:
I. Degenerative
II. Vascular
Multi-Infarct Dementia
Binswanger's Disease
Vasculitides
III. Neoplasia
Primary and metastatic tumors (e.g. frontal lobe, non-dominant temporal lobe,
diencephalon,corpus callosum)
Remote effect of malignancy
IV.Trauma
Non-penetrating head injuries (e.g. dementia pugilistica)
Subdural hematoma
V. Infectious/Inflammatory
VI . Metabolic/Endocrine
VII. Toxic
Alcohol related (e.g. Korsakoff's, Marchiafava-Bignami, multivitamin deficiency, etc.)
Drugs (e.g. anticholinergic, sedatives, hypnotics, anticonvulsants, antihypertensives,
psychotropics, etc.)
Heavy metals
Solvents
poisons
VIII. Hydrocephalus
Communicating
Non-Communicating
X. Pseudo-Dementia
E. EVALUATION:
History, Physical/neurological examination (Hachinski ischemic score - see below)
Mini-mental state examination (screening test - see table below)
Mattis dementia rating scale
Alzheimer's disease assessment scale
Specialized neuropsychological examination
Routine tests
CBC with differential, ESR
Urea, creatinine, calcium, phosphorus, electrolytes, glucose
Liver function tests
Serum B12, Serum Folate
TSH
VDRL
Urinalysis
Chest roentgenogram
ECG
CT scan
Selective Tests
CSF
EEG
MRI
radionuclide cisternography (?)
lumbar infusion testing (?)
HIV
ANA
serum copper, ceruloplasmin, 24-hour urine copper
urine for porphobilinogen, Watson-Schwartz test
serum, peripheral blood leukocytes cultured fibroblasts for enzymatic deficiencies
(arylsulfatases, ceroid lipofuscin, etc.)
Cerebral angiogram
Jejunal biopsy
Brain biopsy
* Scores of greater than 4 indicate a higher probability of Alzheimer's Disease and scores of
7 and above indicate a high probability of multi-infarct dementia.
F. Management:
Team approach (physician, visiting nurse, social worker)
Treat specific causes
Adequate food and fluid intake
Treat intercurrent illness and associated conditions
Environmental therapy (avoid unfamiliar surroundings, challenging situations, frequent
relocations)
Counseling (spouse, caretaker, etc.)
Pharmacotherapy
Antidepressants
Avoid sedatives
Avoid unproven therapies (e.g. cerebral vasodilators, etc.)
Tacrine
£ POLYMYOSITIS AND DERMATOMYOSITIS
B. CLINICAL CLASSIFICATION:
I. Polymyositis of the adult
II. Dermatomyositis of the adult
III. Dermatomyositis of childhood and the young adult
IV. Dermatomyositis associated with connective tissue disorder (scleroderma, SLE, RA,
MCTD, Sjögren)
V. Polymyositis associated with connective tissue disorder (Scleroderma, SLE, RA,
MCTD, Sjögren)
VI. Dermatomyositis associated with malignancy (e.g.: ovary, stomach)
VII. Polymyositis associated with malignancy (e.g.: ovary, stomach)
D. DIAGNOSIS:
Clinical
Enzymes
- CK (MM), Aldolase
- EMG (myopathic changes, increase muscle irritability)
- Muscle biopsy - degeneration, regeneration, inflammatory changes (PM),
perifascicular atrophy, inflammatory changes (DM)
E. TREATMENT
Corticosteroids
Azathioprine
Methotrexate
Plasma exchange
Total Body irradiation
Treatment of underlying malignancy or connective tissue disease
£ MYASTHENIA GRAVIS
B. EPIDEMIOLOGY:
Incidence - 2-5/yr/million population
Prevalence - 43-64/million
F>M among younger patients
M>F among patients older than 50 yrs.
HLA-A1, HLA-B8, DRW3
C. SIGNS AND SYMPTOMS: Onset is often insidious but may be abrupt. Course is
unpredictable, but
most often is fluctuating.
Muscle Weakness
- Ptosis (unilateral, bilateral or alternating)
- Diplopia (variable and made worse with fatigue)
- Facial weakness (myasthenic snarl)
- Palatal/laryngeal weakness
- Dysphagia, inability to clear secretions
- Weak cough
- Proximal limb muscle weakness
- Myasthenic crisis (incidence 12%-20%/mortality 3%)
- Fatigability
- End of day worsening
E. ASSOCIATED DISEASES
SLE, RA, Sjögren's, Polymyositis, Eaton-Lambert
Ulcerative colitis, pemphigus, pernicious anemia
Hyper/hypothyroidism
Thymic hyperplasia (70%)
Thymomas (15%)
F. DIAGNOSTIC TESTS:
Edrophonium (Tensilon)
Prostigmine
Acetylcholine receptor antibodies
EMG studies
- decremental response at 3Hz > 10%
G. SYMPTOMATIC TREATMENT
Anticholinesterase drugs
Corticosteroids
Azathioprine
Cyclosporine
Cyclophosphamide
Thymectomy
Avoid Drugs which may exacerbate MG
C. CRITERIA FOR DIAGNOSIS: (From Asbury AK, Ann Neurol (Suppl) 1-5, 1981)
I. Features Required for Diagnosis
Ý Progressive motor weakness of more than one limb. The degree ranges from minimal
weakness of the legs, with or without mild ataxia, to total paralysis of the muscles of all four
extremities and the trunk, bulbar and facial paralysis, and external ophthalmoplegia.
Ý Areflexia - Universal areflexia is the rule, though distal areflexia with definite
hyporeflexia of the biceps and knee jerks will suffice if other features are consistent.
E. DIFFERENTIAL DIAGNOSIS:
Myasthenia gravis
Botulism
Periodic paralysis
Poliomyelitis
Epidural abscess
Acute intermittent porphyria
Diphtheritic polyneuropathy
Lyme disease
Toxic polyneuropathies
Organophosphate poisoning
Tick paralysis
Critical illness polyneuropathy
Hypophosphatemia
Basilar artery thrombosis
F. DIAGNOSTIC EVALUATION:
EMG, NCS, F-waves (slowing of conduction velocities, prolonged distal latencies, abnormal
late responses)
Blink reflexes (prolonged or absent)
SSEP
CSF (! protein without pleocytosis)
Vital capacity (VC), maximum pulmonary expiration (PEmax), maximum pulmonary
inspiration (Plmax)
G. TREATMENT:
Supportive
Prevention of complications (monitor heart rate, BP, ECG, VC, PE max, Pl max)
Proper positioning
DVT prophylaxis
Fluid and electrolyte balance
Nutritional support
Analgesia
Psychological support
Early recognition and management of respiratory failure
- inability to clear secretions
- VC < 2-3 times predicted tidal volume (VC < 25ml/kg early intubation is associated with
risk of pulmonary complications)
- PEmax < 40 cmH2O
- Plmax < 20 cmH2O
Plasma exchange (shortens the duration and severity of illness if begun within 2 weeks of
the illness)
IvIg
B. EPIDEMIOLOGY:
Incidence - 0.4-1.8/100,000 population
M>F
Clusters in Guam, and Kii Peninsula (Japan)
C. CLINICAL FORMS:
Amyotrophic lateral sclerosis
Progressive muscular atrophy
Progressive bulbar palsy
Primary Lateral Sclerosis (?)
Pseudobulbar palsy
D. CLINICAL COURSE: The usual clinical course is one of steady progression, leading to
death within 3
yrs in approximately 50% of patients. Rare patients may live up to 30 years.
50% die by 3 years
20% die by 5 years
10% die by 10 years
few survive 20 yrs or longer
Muscle atrophy
Fasciculations
Spasticity, hyperactive reflexes, extensor plantar responses
Preserved eye movements (in general)
Preserved intellect (in general)
Preserved bladder function
Preserved autonomic functions
Absent sensory signs (in general)
F. DIAGNOSIS: The diagnosis is made on the basis of the clinical features. There are no
specific paraclinical markers.
EMG/NCS (Widespread denervation)
Muscle biopsy (seldom needed)
CK (may be slightly elevated)
CSF (protein may be elevated)
Endoscopy, barium swallow, ENT evaluation
Vital capacity
ÝOther investigations are obtained to rule out "treatable" causes of motor neuron disease:
ÝMRI or Myelogram of cranio-cervical junction/spinal cord.
- R/O foramen magnum tumors, basilar invagination, narrowed spinal canal,
intra/extramedullary tumors, syringomyelia, cervical spondylosis
ÝUrine/blood for heavy metals
- lead, mercury, tri-ortho-cresyl phosphate, mercury (?)
ÝT4, TSH, Glucose, Calcium, Phosphorus
- r/o hyperthyroidism (proximal weakness and fasciculations)
- r/o hyperinsulinism (pancreatic adenoma)
- r/o hyperparathyroidism
ÝSearch for occult malignancy, dysproteinemia
- ALS and Waldenstrom's macroglobulinemia
- ALS and renal carcinoma
- ALS and lymphoma
ÝTensilon test, AChR antibodies
-r/o myasthenia gravis
ÝNCV's
- r/o inflammatory polyradiculoneuropathy
ÝCK, Muscle bx, EMG
- R/O inflammatory myopathy
£ PERIPHERAL NEUROPATHIES
B. Investigations
History
General Physical Examination
Neurological Examination
Biochemical Tests
EMG/NCS
Nerve Biopsy (selective cases: e.g.: vasculitis, sarcoidosis, leprosy, CIDP)
£ MULTIPLE SCLEROSIS
C. ETIOLOGY:
1. Autoimmune vs. viral (both are not exclusive)
2. Inherited predisposition
a. 5-20% show familial aggregation
b. Association with DW2 and DRW2 in populations with HLA-A3 and HLA-B7
c. HLA-A3 and HLA-B7 much higher in northern caucasians
D. PATHOLOGY:
Gross
1. Foci of demyelination scattered throughout the cortex, brainstem and spinal cord. Optic
nerves and chiasm are often involved, as are the pons and cerebellar white matter.
2. Distribution of plaques - Most conspicuous in the periventricular white matter, about the
angles of the lateral ventricles and beneath the floor of the fourth ventricle. Optic pathways
and spinal cord frequently involved.
Micro
1. Loss of myelin, reduction in number, or even absence of oligodendrocytes. Astrocytosis
may be pronounced. Lymphocytes and plasma cells can be found. Relative preservation
of axons.
E. CLINICAL FEATURES:
Peak ages of onset
women - 20-35 years old
men - 35-45 years old
overall - 30 years old
Clinical Course
remissions and exacerbations
progressive without remissions
acute MS
neuromyelitis optica (Devic's syndrome)
benign
malignant
Disease Severity
"benign disease" - 30%
"malignant course" - 10%
"intermediate course" - 60%
F. DIAGNOSTIC CRITERIA (Shumacher et al. - 1965)
1. Objective abnormalities attributable to CNS dysfunction.
2. Involvement of two or more separate parts of the CNS, either by history or by neurologic
examination.
3. Neurologic examination must reflect predominantly white matter involvement.
4. The involvement must have occurred in two or more episodes separated by a period of
one month, or must have occurred in a step-wise fashion over a period of at least
six months.
5. The manifestations must not be better attributed to some other disease (by a physician
competent in clinical neurology)
G. LABORATORY EVALUATION:
CT - Hypodense lesions in periventricular white matter
- Acute lesions may enhance
- Chronic disease may result in enlarged ventricles
EVOKED POTENTIALS
VEP - 85% abnormal - definite MS
- 58% abnormal - probable MS
- 37% abnormal - possible MS
BAEP - 37% abnormal with BS symptoms/signs
- 21% abnormal without BS symptoms/signs
SSEP - 77% abnormal - definite MS
- 67% abnormal - probable MS
- 49% abnormal - possible MS
CSF ANALYSIS: Lumbar puncture has not been shown to have any adverse effect on the
course of the disease, and it should be performed diagnostically in all suspected cases.
- WBC >5/mm3 in 30%
> 5/mm3 in 5%
>20/mm3 in 1%
>90% of cells are mononuclear
- Protein <55mg/dL in 75%
>100mg/dL unusual
- lgG elevated in 50% of patients
- lgG index increased in 80-90% of patients
- Oligoclonal bands in 70-90% of patients with definite MS
- Oligoclonal bands may still be seen, even when the CSF total IgG is not elevated
- Myelin basic protein elevated in 50% of patients with definite MS
- Free Kappa chains in 85% of patients with definite MS
- Gamma globulin elevated in 75% of patients
H. THERAPY:
1. Symptomatic
Spasticity: Physical Therapy, Baclofen, Diazepam, Dantrolene Sodium, Surgery
Dystonic: Carbamazepine
Spasms: Phenytoin, Baclofen
Episodic Pain: Amitriptyline, Non-Steroidal Anti-Inflammatory Drugs
Trigeminal Neuralgia: Carbamazepine
Seizures: Carbamazepine, Phenytoin
Bladder Dysfunction: Propantheline, Straight Catheter
Cerebellar Tremor; Isoniazid
Depression: Amitriptyline, Desipramine, Imipramine, Supportive Therapy
Emotional: Amitriptyline
Incontinence: L-Dopa, Bromocriptine
Anxiety: Alprazolam, Diazepam
2. Immunomodulators
ACTH Methylprednisolone
Cyclophosphamide Azathioprine
Cyclosporine A Beta Interferon
Poly ICLC Transfer Factor
Copolymer 1 Methisoprinol
Plasmapheresis Lymphoctyopheresis
Antilymphocyte Globulin Total Lymphoid Irradiation
£ CHOREATIC DYSKINESIAS
B. CLASSIFICATION:
Huntington's disease Sydenham's
Chorea gravidarum Hereditary chorea with Acanthocytosis
Familial Paroxysmal Choreoathetosis Benign Hereditary Chorea
Senile Chorea Drug Induced Chorea
Systemic lupus erythematosus - oral contraceptives
Polycythemia Vera - neuroleptics
Stroke (basal ganglia infarcts) - phenytoin
Trauma - levodopa
Hyperthyroidism
Hyponatremia
Subdural hematoma
Post-thalamotomy
C. HUNTINGTON'S DISEASE
autosomal dominance, complete penetrance
defect localized to short arm of chromosome 4
prevalence - 5-10 cases per 100,000 population
age of onset 35-42 years
Onset before age 15 (Westphal variant)
- akinetic rigid presentation
- seizures
- intellectual decline
Mean duration of illness 10-25 years
Clinical/Paraclinical Studies
- chorea (chorea initially, followed by dystonia)
- dementia
- altered behavior
- increase risk of suicide
- CT/MRI: caudate atrophy, hydrocephalus "ex-vacuo"
- PET: decreased striatal glucose metabolism
D. MANAGEMENT:
Anti-dopaminergic agents
- phenothiazines, butyrophenones, thioxanthenes
- reserpine, tetrabenazine
Psychological Support
Antidepressants (e.g.: tricyclics)
Genetic counseling
B. EPIDEMIOLOGY:
Common
Prevalence 1 per 1000 population
Incidence 20 cases per 100,000 individuals per year
Middle age and elderly
C. CLASSIFICATION:
Primary (Parkinson's Disease)
Secondary
- Drug induced (phenothiazines, butyrophenones, thioxanthines, metoclopramide,
reserpine, alpha-methyldopa)
- Infectious (post-encephalitic, lues, Jakob-Creutzfeld)
- Toxin (MPTP, carbon monoxide, carbon disulfide, manganese, cyanide)
- Metabolic (Wilson's disease, Hallevorden-Spatz disease, parathyroid dysfunction)
- Structural (basal ganglia tumors, repeated head injury)
- Vascular (?)
- Parkinsonism Plus (Progressive supranuclear palsy, Shy-Drager, olivo-ponto-cerebellar
degeneration, striato-nigral degeneration, corticospinal degeneration with neuronal
achromasia, Parkinson-dementia complex of Guam, Parkinsonism with amyotrophy)
F. MANAGEMENT:
Anticholinergic Drugs, Amantadine, Levodopa + Peripheral Dopa Decarboxylase Inhibitor
(Sinemet 10:100, Sinemet 25:100, Sinemet 25:250), Sinemet CR (Levodopa,
200mg/carbidopa 50mg), Bromocriptine, Other Dopamine Agonists (e.g.: Pergolide,
Lisuride), MAO-B Inhibitors (Deprenyl), Antioxidants (Alpha Tocopherol),
Antidepressants
(Amitriptyline, Trazodone, Fluoxetine), Beta Blockers (for action tremor if present), Brain
Grafts (?), Physical Therapy
£ TREMOR
B. Classification
Resting (3.5 - 7 Hz)
- Parkinsonism
Postural (6 -11 Hz)
-Physiological tremor
- Anxiety
- Hyperthyroidism
- Alcohol
-Hypoglycemia
-Sympathomimetic
- Lithium Carbonate
- Tricyclics (imipramine, amitriptyline)
- Benign (essential) Tremor (may be familial)
Intention (3 - 7 Hz)
- Brainstem/Cerebellar Disease (e.g.: multiple sclerosis, spino-cerebellar degeneration)
C. Management
Resting Tremor
- D/C offending drugs (e.g.: neuroleptics)
- Rx for Parkinson's disease
Postural Tremor
- Rx underlying cause
- D/C offending drug (e.g.: lithium, imipramine)
- Beta blockers (essential tremor)
- Primidone (essential tremor)
Intention Tremor
- Rx underlying cause
£ MYOCLONUS
B. Classification
Generalized
Symptomatic
a. Myoclonus in idiopathic epilepsy
b. Progressive myoclonic epilepsy
Familial myoclonic epilepsy (Unverricht-Lundberg disease)
Myoclonus associated with spinocerebellar degeneration
Classification (Cont)
(Ramsay-Hunt syndrome)
Lafora body disease
GM2 gangliosidosis (Tay-Sach's disease)
Ceroid lipofuscinosis (Batten's disease)
Sialidosis (cherry red spot myoclonus syndrome)
C. Others
Encephalitis lethargica
Jakob-Creutzfeld disease
Alzheimer's disease
Subacute sclerosing panencephalitis
Infantile myoclonic encephalopathy
Post cerebral anoxia (Lance-Adams syndrome)
Hepatic, renal respiratory failure
Hyponatremia, hypocalcemia
Withdrawal of addictive drugs (e.g.: alcohol)
Benign essential (familial) myoclonus
Focal (segmental)
Symptomatic
Subacute myoclonic spinal neuronitis
Spinal cord tumors, infarcts, trauma
Others
Palatal myoclonus
Hemifacial spasm
Cortical reflex myoclonus
Epilepsia partialis continua
£ DYSTONIA
B. Classification
Generalized Symptomatic
Encephalitis lethargica Wilson's disease
Lesch-Nyham disease Juvenile Huntington's disease
Hallevorden-Spatz disease Athetoid cerebral palsy
Ataxia telangiectasia Mitochondrial encephalopathies
Drug related (e.g.: neuroleptics) Idiopathic
Dystonia musculorum deformans Autosomal recessive
Sex-linked recessive Autosomal dominant
Sporadic Paroxysmal
Paroxysmal kinesogenic choreoathetosis Paroxysmal dystonic choreoathetosis
Dystonia with diurnal fluctuations Focal (segmental)
Symptomatic Post-hemiplegic dystonia
Classification (CONT)
C. Management
Levodopa, Carbamazepine, Benzodiazepines, Anticholinergics, Tetrabenazine,
Phenothiazines (?), Butirophenones (?), Sterotaxic surgery.
£ TICS
B. Classification
Simple Tics
transient tic of childhood
chronic simple tic
Complex Multiple Tics
Gilles de la Tourette syndrome
ages 5 - 15 years
mode of transmission - major semidominant gene
waxing waning course
duration greater than 1 year
multiple or single tics
vocalizations
copropraxia
echopraxia
echolalia
coprolalia
Rx: haloperidol, pimozide, clonidine, tetrabenazine
Chronic multiple tics
Symptomatic Tics
Encephalitis lethargica
Drug induced tics
Posttraumatic
Neuro-acanthocytosis
£ NEUROLOGICAL COMPLICATIONS OF ALCOHOLISM
1. ALCOHOLIC INTOXICATION
< 50 mg/dl - mild incoordination
400-700 - coma, respiratory depression, death (Rx of ethanol induced coma is supportive)
- R/O ethanol induced hypoglycemia, alcoholic ketoacidosis, or ethylene glycol,
methanol or isopropanol intoxication
2. WITHDRAWAL SYNDROMES
A. Minor Syndrome
Stage I
a) onset 6-24 hours after reduction of intake
b) adrenergic overdrive
c) 25% progress to Stage II; 10% progress to Stage III
Stage II
a) Stage I symptoms, plus visual, auditory and tactile hallucinations
Stage III (withdrawal seizures or rum fits)
a) onset 7-48 hours after cessation of drinking (50% occur 13-24 hours after drinking has
ceased)
b) generalized tonic-clonic seizures
c) single in 40% of cases
d) status epilepticus in 4% of cases
e) normal interictal EEG. Heightened photoconvulsive response
B. Major Syndrome (Delirium Tremens)
Stage IV (early presentation (24 hrs), or late presentation (12 days)
a) marked adrenergic overdrive
b) frank hallucinations
c) 5-15% mortality
C. Management
Stages I-II Thiamine
Multivitamins
Folate
Chlordiazepoxide, Clorazepate, Oxazepam, Diazepam
Propranolol, Atenolol
Clonidine
Stage III Most alcohol withdrawal seizures do not require pharmacologic
intervention
Stage IV Intensive care
Treatment as in Stages I-II-III
Support vital signs and volume status
Restrain patient
hypothermia
Management: Thiamine, bed rest, nutritional supplements
B. Korsakoff Amnestic Syndrome
anterograde amnesia
retrograde amnesia
intact immediate recall
confabulation (not essential for dx)
loss of insight
C. Alcoholic Amblyopia
D. Pellagra
Dementia
Dermatitis
Diarrhea
Neuropathy
E. Peripheral Neuropathy
axonal damage
segmental demyelination
8. OTHER ASSOCIATIONS
A. Vitamin B-12 Deficiency
B. Hypoglycemia
C. Stroke
D. Movement Disorders
A. Classification:
I Tumors of Glial Origin
Astrocytoma (Grades I-IV)
Oligodendroglioma
Ependymoma
C. Evaluation:
History
General Physical Examination
Neurological Examination
Neuro-Ophthalmology Evaluation
CT without and with intravenous contrast
MRI without and with gadolinium
EEG
Cerebral angiography (seldom needed)
Endocrine evaluation of pituitary and hypothalamic activity (if indicated)
Blood count, biochemical profile, urinalysis
Chest roentgenogram
Bone scan, IVP, GI series, bone marrow, CT of chest/abdomen (if indicated)
D. Management:
Anticonvulsants
Corticosteroids
Surgery (conventional, stereotactic)
Radiation therapy
Chemotherapy
Dopamine agonists (e.g.: bromocriptine " prolactinomas)
B. PATHOPHYSIOLOGY:
1. Diffuse, bilateral cerebral hemispheric lesions
2. Dysfunction of brainstem reticular activating mechanism
Irregular breathing
Cheyne-Stokes Bilateral dysfunction
(hemispheres, diencephalon)
Central Neurogenic Low midbrain, upper pontine
Hyperventilation lesion
Apneustic Breathing Mid-caudal pontine lesion
Ataxic Breathing Medullary lesion
b. Blood Pressure
Arterial Hypertension Reflex response to increase intracranial pressure
Chronic hypertension
Arterial Hypotension Hypovolemia
Hemorrhagic shock
Myocardial depression
c. Temperature
Hyperthermia Meningitis
Encephalitis
Sepsis
Disruption of autonomic pathways
Hypothermia Hypoglycemia
Hypothyroidism
Wernicke's encephalopathy
Sedative overdose
Environmental exposure
d. Meningismus Meningitis
Subarachnoid hemorrhage
Tonsillar herniation
e. Pupils: The pupillary pathway is relatively resistant to metabolic insults. The presence
or absence of the pupillary light reflex is the single most important physical sign
distinguishing structural from metabolic coma (e.g.: metabolic cause of coma - small and
reactive
pupils).
Diencephalic - small, reactive
Tectal - large "fixed", hippus
Midbrain - midposition fixed
III Nerve (Uncal Herniation) - fixed and dilated
Pontine - pinpoint with preserved light reflex (use magnifying glass)
f. Ocular Motility: Most comatose patients have their eyes closed by tonic contractions of
orbicularis oculi
Oculocephalic reflex (Doll's headeye phenomenon) - Vestibular portion of CN VIII
Oculovestibular reflex (Caloric response) - Connection of brainstem pathways with CN III,
IV and VI
Spontaneous eye movements
- Periodic alternating gaze
- repetitive divergence
- Ocular bobbing and varieties
- Nystagmoid jerking of a single eye
- Vertical ocular myoclonus
E. HERNIATION SYNDROMES
« Cyngulate herniation syndrome
« Uncal herniation syndrome
« Central herniation syndrome
« Downward cerebellar tonsillar herniation syndrome
« Upward cerebellar herniation syndrome
Flexion - withdrawal 4
Flexion - abnormal 3
(decorticate rigidity)
Extension 2
(decerebrate rigidity)
No response 1
The Glasgow Coma Scale, based upon eye opening, verbal and motor responses, is a
practical means of monitoring changes in level of consciousness. If response on the scale
is given a number, the responsiveness of the patient can be expressed by summation of the
figures. Lowest score is 3; highest is 15.
G. GENERAL MEASURES
Airway__________clear airway
Breathing__________intubation?
Circulation__________Vasopressors?
CBC with diff, glucose, lytes, liver enzymes, metabolic/toxicologic screen
if persistent seizures
Barbiturate Coma
General Anesthesia
Fluid balance and alimentation
Control of body temperature
Representative Disorders
Tumors, Anoxia, Infarcts Obstructive
Abscess, Brain Death hydrocephalus
Hematoma, Water intoxication
Infarcts
Lead Encephalopathy,
Purulent Meningitis
Therapeutic Effects
Corticosteroids + - - -
Ventricular Shunt - - - +
C. Intracranial Pressure - (ICP): The cranial cavity contains three constituents: brain
substance (including interstitial fluid), cerebrospinal fluid (CSF), and blood volume
(arterial
and venous blood).
CPP (MABP-ICP).
Remediable factors likely to increase ICP (e.g.: straining and coughing, obstruction to
venous drainage, hypoxia, hypercapnia, hyperthermia, seizures, arterial hypertension,
hyponatremia, etc) should be corrected. Differentiate tissue shifts from jeobally increased
ICP.
Optimal Head Positioning
15 - 30 degree head up
neutral position
elevation of torso may decrease CPP
Hyperventilation
PaCo225 torr (some favor moderate hypocapnia: 35-38 torr).
minimize noxious stimuli of intubation; maintain mormoxia (SpO2>95%)
(e.g.: xylocaine, pentothal)
Fluid Restriction
2/3 maintenance
avoid hypotonic solutions
Osmotic Agents - Mannitol (20% solution), Glycerol (10% solution)
Mannitol 0.75 - 1 g/Kg IV over 15 minutes (bolus)
0.25 g/Kg q 4 hours
dosage according to clinical response, serum osmolality, ICP
Serum osmolality should not exceed 320m Osm because of risks of dehydration, renal
failure,
acidosis. Avoid hypernatremia.
replace fluid lost by diuresis (crystalloids)
maintain circulatory normovolemia
adequate glucose control (150 - 250 mg/100 ml)
Non Osmotic Diuretics
Furosemide (0.3 - 0.7 mg/Kg)
Ethacrynic Acid
Acetazolamide
Others
Corticosteroids (see B for indications)
Barbiturates
CSF Drainage
Surgery (e.g.: hemicraniectomy; skull vault removal).
Extramedullary Lesions
Primary Tumors (e.g.: meningioma, neurofibroma)
Metastatic Tumors (e.g.: metastatic carcinoma, lymphoma, sarcoma, neuroblastoma)
Pyogenic Epidural Infections
Intramedullary Lesions
Tumors (e.g.: astrocytoma, ependymoma, metastasis)
Intramedullary Abscess
Vascular (e.g.: hematomyelia, angioma)
The most common tumors to metastasize to the spine are breast, lung and prostate,
although unknown primary tumors occur in up to 10% in some series (Table 2).
B. Clinical Presentation: Depending on the cause, the array of symptoms and signs may
vary,
but the development of extrinsic spinal cord compression usually follows a sequential
pattern.
1. Spinal Pain
2. Radicular Pain
3. Weakness
4. Sensory Loss
5. Reflex Changes
6. Disorders of Sphincters
C. Diagnostic Evaluation: Once the diagnosis of spinal cord compression is suspected, the
following should be obtained. The diagnostic procedure of choice nowadays is without
question MRI of the spine. MRI allows one not only to find the extent of the lesion but also
allows one to examine for multiple lesions.
Roentgenogram of the spine
Myelography
CT scan of the spine
MRI of the spinal cord
D. Treatment
High dose steroids (reduce spinal cord edema, oncolytic effect)
Radiation Therapy
Surgical Decompression
resectable lesion
unknown primary
already undergone maximum RT
deterioration while on RT
Chemotherapy
Diagnostic and Therapeutic Algorithm for Patients with Spinal Cord Compression
Dexamethasone g