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EBook Blueprints Neurology Blueprints Series Fifth Edition PDF Docx Kindle Full Chapter
EBook Blueprints Neurology Blueprints Series Fifth Edition PDF Docx Kindle Full Chapter
Preface
Acknowledgments
Abbreviations
2 Neurologic Investigations
4 Neuro-Ophthalmology
12 Dementia
13 Sleep Disorders
14 Vascular Disease
15 Seizures
16 Movement Disorders
17 Head Trauma
25 Pediatric Neurology
Questions
Answers
Appendix: Evidence-Based Resources
Index
Preface
B lueprints Neurology was first published more than 15 years ago as one of
a series of books designed to help medical students prepare for USMLE
Steps 2 and 3. As the study and practice of Medicine and Neurology and
professional board examinations have evolved over the years, so too has
Blueprints Neurology changed to assist students to learn Neurology in
multiple evaluation and examination settings.
Examination preparation remains at the core of the series. To that end, the
authors review the subject matter of the examination before each edition. The
authors and editors work together to organize the most important, current,
and factually correct material into a complete but concise review guide. Our
goal remains integrating the depth of factual knowledge with the breadth of
practice information in order to optimize both understanding and retention.
We have been pleased to hear from our readers that the book is utilized by
many medical students during their clinical rotations, as well as in
preparation for shelf and board examinations. Residents in Internal Medicine,
Emergency Medicine, and Family Practice, as well as nurse practitioners and
physicians’ associates have found Blueprints helpful during the Neurology
portion of their training. We believe the book’s applications have broadened
with each edition due to the quality of our authors’ experience and their
dedication to highlighting and clarifying a targeted range of basic but
important topics that should be mastered.
Virtually all chapters are authored by experts in the content area, including
neurologists from both academic medicine and busy private practice groups.
They have incorporated suggestions received from medical students, faculty,
clinicians, and program directors with regard to content and organization.
Each chapter covers a single subject area for review; most can be read in
under an hour. “Key Points,” highlighted throughout, facilitate quick review
of the key concepts tested most frequently. Most chapters have Vignettes
with characteristic clinical presentations to test one’s review of the chapter.
The 100 Questions at the end of the book are also written in the “Clinical
Vignette” style used in USMLE and Board examinations, as recommended
by student reviewers of the Blueprints series.
This fifth edition of Blueprints Neurology is the most thoroughly updated
edition to date, with several new authors (generally closer in stage of
education to the students for whom the book is written). It includes important
updates on areas such as Multiple Sclerosis, Stroke, Epilepsy, Movement
disorders and their genetic bases, Sleep disorders such as narcolepsy and
cataplexy, and new drug and other treatments for each. Each chapter includes
the most recent information and practice principles available and accepted at
the time of publication.
We hope that readers of Blueprints Neurology will come to see the wonder
of the human nervous system, how important it is to individuals in health and
when it fails, and how the study and practice of Neurology is more helpful to
patients than ever before.
Frank W. Drislane, MD
Aimee K. Boegle, MD, PhD
Alexandra Hovaguimian, MD
Courtney McIlduff, MD, MMSc
Andrew W. Tarulli, MD
Louis R. Caplan, MD
Acknowledgments
W e thank our patients for the opportunity of working with them and
learning Neurology; our colleagues and teachers (and particularly,
upon his retirement, Michael Ronthal, MBBCh) at Beth Israel Deaconess
Medical Center Neurology department for teaching us more fascinating
concepts about the nervous system; and our families for tolerating the many
hours spent writing and revising this book.
Abbreviations
A(β) amyloid-beta
ABP abductor pollicis brevis
Abs antibodies
AβPP amyloid–beta protein precursor
ACA anterior cerebral artery
ACE angiotensin-converting enzyme
AChR acetylcholine receptor
AD Alzheimer disease
ADEM acute disseminated encephalomyelitis
ADHD attention deficit–hyperactivity disorder
ADM abductor digiti minimi
AICA anteroinferior cerebellar artery
AIDP acute inflammatory demyelinating polyradiculoneuropathy
AIDS acquired immunodeficiency syndrome
AION anterior ischemic optic neuropathy
ALS amyotrophic lateral sclerosis
ANA antinuclear antibody
APP amyloid precursor protein
APS antiphospholipid syndrome
ASD anti-seizure drug
AVM arteriovenous malformation
AZT zidovudine
BMD Becker muscular dystrophy
BPPV benign positional paroxysmal vertigo
CBC complete blood count
GMP cyclic guanosine monophosphate
CIDP chronic inflammatory demyelinating polyradiculoneuropathy
CJD Creutzfeldt-Jakob disease
CK creatine kinase
CMAP compound muscle action potential
CMT Charcot-Marie-Tooth disease
CN cranial nerve
CNS central nervous system
COMT catechol O-methyl transferase
CP cerebral palsy
CPAP continuous positive airway pressure
CSF cerebrospinal fluid
CT computed tomography
DH detrusor hyperreflexia
DI detrusor instability
DLB dementia with Lewy bodies
DM dermatomyositis
DMD Duchenne muscular dystrophy
DSD detrusor sphincter dyssynergia
DTRs deep tendon reflexes
DWI diffusion-weighted imaging
EA episodic ataxia
ED erectile dysfunction
EEG electroencephalogram
EMG electromyography
ER emergency room
ESR erythrocyte sedimentation rate
ET essential tremor
EWN Edinger-Westphal nuclei
FDI first dorsal interosseus
FEV1 forced expiratory volume in 1 second
FLAIR fluid-attenuated inversion recovery
FTA fluorescent treponemal antibody
FTD frontotemporal dementia
FVC forced vital capacity
GAD glutamic acid decarboxylase
GBS Guillain-Barré syndrome
GCS Glasgow Coma Scale
GTC generalized tonic–clonic
HD Huntington’s disease
HIV human immunodeficiency virus
HNPP hereditary neuropathy with liability to pressure palsies
HS Horner’s syndrome
HSAN hereditary sensory and autonomic neuropathy
HSV herpes simplex virus
IBM inclusion body myositis
ICA internal cerebral artery
ICP intracranial pressure
ICU intensive care unit
IIH idiopathic intracranial hypertension
INO internuclear ophthalmoplegia
INR international normalized ratio
IVIg intravenous immunoglobulin
LEMS Lambert-Eaton myasthenic syndrome
LGN lateral geniculate nucleus
LMN lower motor neuron
LND light-near dissociation
LP lumbar puncture
MAG myelin-associated glycoprotein
MCA middle cerebral artery
MELAS mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke
MERRF myoclonic epilepsy with ragged red fibers
MFS Miller Fisher syndrome
MG myasthenia gravis
MLF medial longitudinal fasciculus
MMN multifocal motor neuropathy
MND motor neuron disease
MRA magnetic resonance angiography
MRC Medical Research Council
MRI magnetic resonance imaging
MRV magnetic resonance venography
MS multiple sclerosis
MSA multiple system atrophy
MSLT multiple sleep latency test
MuSK muscle-specific kinase
NCS nerve conduction studies
NCV nerve conduction velocity
NFTs neurofibrillary tangles
NIF negative inspiratory force
NMDA N-methyl-D-aspartate
NMJ neuromuscular junction
NMS neuroleptic malignant syndrome
NSAIDs nonsteroidal anti-inflammatory drug
OCD obsessive-compulsive disorder
ON optic neuritis
PANDAS pediatric autoimmune neurologic disorders associated with streptococcal
infection
PAS periodic acid–Schiff
PCA posterior cerebral arteries
PCD paraneoplastic cerebellar degeneration
PCNSL primary central nervous system lymphoma
PCR polymerase chain reaction
PD Parkinson’s disease
PDC paroxysmal (nonkinesigenic) dystonic choreoathetosis
PEO progressive external ophthalmoplegia
PET positron emission tomography
PICA posteroinferior cerebellar artery
PKC paroxysmal kinesigenic choreoathetosis
PM polymyositis
PML progressive multifocal leukoencephalopathy
PN peripheral neuropathy
PNS peripheral nervous system
POTS postural orthostatic tachycardia syndrome
PP periodic paralysis
PPD purified protein derivative
PPRF paramedian pontine reticular formation
PS1 presenilin 1
PS2 presenilin 2
PSP progressive supranuclear palsy
PT prothrombin time
PTT partial thromboplastin time
PVR postvoid residual
QSART quantitative sudomotor axon reflex test
RAPD relative afferent pupillary defect
REM rapid eye movement
RF radiofrequency
riMLF rostral interstitial nucleus of the MLF
RPR rapid plasma reagin
rt-PA recombinant tissue-type plasminogen activator
SAH subarachnoid hemorrhage
SCA spinocerebellar ataxia
SCA superior cerebellar artery
SE status epilepticus
SLE systemic lupus erythematosus
SMA spinal muscular atrophy
SNAP sensory nerve action potential
SPECT single-photon emission computed tomography
SSRI selective serotonin reuptake inhibitor
STT spinothalamic tract
TB tuberculosis
TCD transcranial Doppler
TE time to echo
TIA transient ischemic attack
TORCH toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex
TR time to repetition
TSC tuberous sclerosis complex
UMN upper motor neuron
VA visual acuity
VDRL Venereal Disease Research Laboratory
VOR vestibulo-ocular reflex
VP venous pulsation
VPL ventroposterolateral
WD Wilson’s disease
PART I BASICS OF NEUROLOGY
1 The Neurologic Examination
The care of patients in all specialties has been enhanced by the use of an
increasingly sophisticated array of biomarkers, genetic tests, and imaging
modalities. Yet even in the setting of these critical advancements, the
physical examination remains of utmost importance in Neurology. We glean
valuable information from listening to the manner in which concerns are
expressed, observing how patients walk into the clinic or lie in a hospital bed,
and performing maneuvers designed to interrogate the functional integrity of
nervous system components. Ultimately, the examination is a tool we use to
pinpoint the nature and origin of abnormalities. The resultant picture can
narrow the list of possible diagnoses and guide further investigation.
PRINCIPLES
1. It is useful to conduct a complete examination at least once for every
Neurology patient. The neurologic examination may be unique in its
length, but it is worthwhile to complete a thorough assessment at least
once with each Neurology patient for several reasons. First, that
examination provides a baseline assessment of neurologic status—which
can be particularly valuable in the hospital, where examinations can
evolve in important and sometimes unforeseen ways. Second, a full
examination may uncover unexpected abnormalities. One might be
tempted to skip a full mental status examination for a patient who can
exchange pleasantries normally—only to be surprised when the patient
identifies the year as 1962. Because neurologic problems can present with
discrete deficits, formal testing in each domain is sensible. Third,
abnormalities on basic tests can point out the need for more in-depth,
specialized evaluations. For example, the emergence of diplopia on testing
extraocular movements might prompt a search for fatigable eyelid
weakness that can raise concern for myasthenia gravis. In this way, the
neurologic examination becomes tailored for each individual patient.
Fourth, the examination allows one to directly confirm or refute
hypotheses about contributory problems suggested by the history. Foot
drop is more likely to result from a lumbosacral radiculopathy if
accompanied by back pain; a positive straight leg raise test can help
corroborate this explanation. Finally, the examination can show a pattern
of abnormalities that provides a clue as to where in the nervous system the
problem lies.
2. The goal is to localize the problem. The nervous system is extensive.
Broadly, we can characterize elements as central or peripheral. The central
nervous system includes the brain and spinal cord. The peripheral nervous
system (PNS) incorporates nerve roots, plexi, peripheral nerves,
neuromuscular junctions, and muscles. Dysfunction originating from each
of these locations can translate into distinctive examination findings
(Table 1-1); recognizing characteristic patterns is often the key to
localizing a deficit. Using this approach, the exam can help determine
whether left hand weakness stems from carpal tunnel syndrome, a brachial
plexus injury, cervical radiculopathy, or a middle cerebral artery stroke.
These distinctions are important because the diagnostic steps, prognoses,
and therapies differ for each of these conditions.
3. Findings should be interpreted in the context of the history. In
performing a comprehensive neurologic examination, it is not uncommon
to detect incidental abnormalities. Particularly at the start of one’s career,
it can be difficult to discern whether certain abnormalities are important.
One should assign greater weight to findings related to the presenting
symptoms or a patient’s medical history. For instance, abnormal sensation
in a football-shaped region over the anterolateral thigh may be a key
finding in an obese person who developed burning in this area after
wearing tight-fitting pants, but an unimportant (or untrustworthy)
discovery in an individual who presents with an acute change in mental
status.
KEY POINTS
● A complete neurologic examination is important to identify and characterize patterns of
abnormalities.
● The goal of the examination is to localize lesions in the nervous system.
● Findings should always be interpreted in the context of the clinical history.
MENTAL STATUS
The mental status exam is performed to identify cognitive deficits related to
specific regions in the brain. The first step is to assess level of consciousness,
which can range from awake and alert to unarousable even with noxious
stimulation. Rather than using medical terms such as stuporous or obtunded
in the latter setting, it is more helpful to describe what external stimuli are
required to arouse a patient or maintain wakefulness. The level of
consciousness frames further testing of cognitive function. Attention is
tested, typically by asking patients to recite spans of numbers, months, or
words such as “world,” forward and backward. A specific form of inattention
is referred to as neglect. Patients with dense neglect may fail to describe
items on one side of a picture or of their surroundings or fail to bisect a line
properly. Subtle neglect may manifest as extinction to double simultaneous
stimulation; in this scenario, a patient can sense a single visual or sensory
stimulus on either side of the body but reports it on the nonneglected side
alone when bilateral stimuli are presented. In some cases, it is not possible to
perform formal tests of attention because patients become focused on one
detail or task and keep repeating it (“perseveration”). Deficits in attention are
important to recognize because they can compromise the ability to complete
other tasks in the mental status examination. Orientation is tested by asking
a patient to identify his or her name and location as well as the day, date,
month, year, and current situation.
Memory is assessed by asking patients to repeat several words
immediately and again after intervals (e.g., 30 seconds and 3 minutes). The
examiner should make note of whether the patient is aware of current events.
Language is assessed in several ways: by listening to the fluency and
prosody of spontaneous speech, identifying word substitutions (i.e.,
paraphasic errors), and assessing the ability to repeat phrases, read, write, and
name common and uncommon objects. Furthermore, the examiner can ask
the patient to name as many words as possible starting with the letter “F,”
“A,” or “S” in 1 minute, paying attention not only to the number of words
generated but also to the manner in which they are named. For example, does
the patient recognize whether she or he repeated words? Were words
volunteered in identifiable categories? In addition to insight into language
function, these details provide insight into how well patients can plan and
organize information (i.e., frontal lobe executive function). To assess verbal
comprehension, check to see if patients can follow spoken midline,
appendicular, and cross-body commands.
Other tests of frontal lobe function include learning and then repeating a
simple motor sequence of hand postures (i.e., the Luria manual sequencing
task). Another test of appropriate inhibition, the go/no go test, comprises
tapping the table when only one letter (e.g., “B”) is said aloud in a string of
letters. Perseveration is also considered a frontal deficit. If cognitive
impairment emerges as a concern, the examiner should consider looking for
the presence of primitive reflexes, which are signs of “frontal release” or
disinhibition. Examples include the palmo-mental, snout, and rooting
reflexes; of note, the examiner should be careful not to overinterpret these
reflexes, because they can occur in normal subjects with age or may not be
relevant to the presenting problem.
KEY POINTS
● The mental status exam should begin with assessment of level of consciousness and attention
because these can affect the interpretation of subsequent tests.
● Memory, language, calculation, praxis, visuospatial, and frontal lobe function are other key
elements of the mental status exam that can suggest focal brain lesions.
CRANIAL NERVES
One way to test cranial nerves is to start at eye level and move down the face
in approximate numerical order (Table 1-3).
Olfaction (I) is rarely tested. When patients report alterations in the ability
to smell, each nostril should be tested separately. A non-noxious stimulus,
such as coffee or vanilla, can be used.
Optic nerve (II) function is assessed in several ways. Visual acuity is
investigated with a near card. Visual fields are tested by having the patient
cover one eye and focus on the examiner’s nose; they are then asked to signal
when they can appreciate a small red object enter the field of view from each
of four quadrants when the object is held halfway between the patient’s eye
and the examiner’s (the limits of the patient’s visual fields should correspond
to those of the examiner’s). Direct visualization of the optic nerve can be
achieved by fundoscopy. The afferent limb of the pupillary light reflex is also
mediated by the optic nerve; the efferent limb is subtended by CN III.
Extraocular movements (III, IV, and VI) are tested in three main ways: by
having the patient pursue a moving target (e.g., an examiner’s finger drawing
of the letter “H” in front of the face; i.e., pursuit); by directing the patient’s
gaze to various stationary targets or directions (saccades); and by having the
patient fixate on an object while the head is turned passively (vestibulo-
ocular movements). The presence of nystagmus should be noted.
Muscles of mastication (V) are tested by assessing the strength of jaw
opening and palpating the contraction of the masseter when the jaw is
clenched. Facial sensation can be tested to all modalities over the forehead
(V1), cheek (V2), and jaw (V3) regions. The afferent limb of the corneal
reflex is mediated by CN V; the efferent limb is controlled by CN VII.
Muscles of facial expression (VII) are tested by having patients raise the
eyebrows, squeeze the eyes shut, puff the cheeks, or show the teeth. Though
uncommonly tested, taste over the anterior two-thirds of the tongue is
mediated by this nerve and can be evaluated with sugar or another
nonnoxious stimulus.
Hearing (VIII) may be evaluated in each ear simply by whispering or
rubbing fingers; more detailed assessment of hearing loss may be
accomplished with the Weber and Rinne tuning fork (512 Hz) tests.
Vestibular function can be tested in many ways, including evaluation of eye
fixation while the patient’s head is turned rapidly or by observation for a
gradual rotation of gait direction while the patient is walking in place with the
eyes closed.
TABLE 1-3. The Cranial Nerves
Nerve Name Exit through the Function
Skull
I Olfactory Cribriform plate Olfaction (test using nonnoxious
substance)
II Optic Optic canal Vision (acuity, fields, color), afferent
limb of pupillary reflex
III Oculomotor Superior orbital fissure Superior rectus, inferior rectus, medial
rectus, inferior oblique, levator
palpebrae, efferent limb of pupillary
reflex
IV Trochlear Superior orbital fissure Superior oblique of contralateral eye
V Trigeminal Superior orbital fissure Muscles of mastication, tensor
(V1), foramen tympani, tensor veli palatini, facial
rotundum (V2), sensation, afferent limb of corneal
foramen ovale (V3) reflex
VI Abducens Superior orbital fissure Lateral rectus
VII Facial Internal auditory Muscles of facial expression, stapedius,
meatus taste on anterior two-thirds of tongue,
efferent limb of corneal reflex
VIII Vestibulocochlear Internal auditory Hearing, vestibular function
meatus
IX Glossopharyngeal Jugular foramen Movement of palate, sensation over
palate and pharynx, taste over posterior
one-third of tongue, afferent limb of
gag reflex
X Vagus Jugular foramen Movement of palate; sensation over
pharynx, larynx, and epiglottis; efferent
limb of gag reflex; parasympathetic
function of viscera
XI Accessory Jugular foramen Sternocleidomastoid and trapezius
movement
XII Hypoglossal Hypoglossal foramen Tongue movement
Palate elevation should be symmetric, and the voice should not be hoarse
or nasal (IX and X). Failure of the right palate to elevate implies pathology of
the right glossopharyngeal nerve. The gag reflex is also mediated by these
nerves.
Sternocleidomastoid strength is tested by having the patient turn the head
against resistance; weakness on turning to the left implies a right accessory
nerve (XI) problem. The trapezius muscle is tested by having patients shrug
the shoulders.
Tongue protrusion should be in the midline. If the tongue deviates toward
the right, the problem lies with the right hypoglossal nerve (XII).
KEY POINTS
● Cranial nerve testing is most easily performed and recorded in approximate numerical order.
● Key elements of the cranial nerve exam include assessment of vision and eye movements,
facial movement and sensation, and movements of the palate and tongue.
MOTOR EXAM
First, bulk is assessed by observing and palpating the muscles and comparing
each side to the other and the patient’s overall muscle bulk to that expected
for age.
Tone is one of the most important parts of the motor exam. In the arms,
tone is checked by moving the patient’s arm, flexing and extending at the
elbow, moving the wrist in a circular fashion, and pronating and supinating
the forearm rapidly using a handshake grip. Abnormalities of tone such as
spasticity and rigidity are discussed in subsequent chapters. Tone in the legs
can be tested well only with the patient supine. The examiner lifts the leg up
suddenly under the knee; in the presence of increased tone, the heel comes
off the bed. Increased tone can be characterized further as rigid or spastic. In
rigid limbs, the examiner can sense increased resistance throughout the
passive movements, but spasticity is speed dependent, with abnormalities
emerging with quick movements (e.g., elbow extension).
Strength is assessed by both observation and direct confrontation (Fig. 1-
2). A pronator drift may be observed in an arm held supinated and extended
in front of the body. The patient may be asked to rise from a chair without
using the arms or to walk on the heels and toes. The power of individual
muscles as assessed by direct confrontation testing is most often graded
according to the Medical Research Council (MRC) scale (Table 1-4). In some
settings, such as the intensive care unit, it is not possible to perform detailed
motor assessments. In this case, the examiner can look to see if there is
symmetry to voluntary limb movements. Another approach is to evaluate
whether the patient can withdraw meaningfully (i.e., pull the examined limb
away from a mildly noxious stimulus such as a pinch).
FIGURE 1-2. Power testing of individual movements. For each movement, the predominant
muscle, peripheral nerve, and nerve root are given. (Reproduced with permission from Ginsberg L.
Lecture Notes: Neurology. 8th ed. Oxford: Blackwell Publishing; 2005:40–41.)
REFLEXES
Muscle stretch (or “deep tendon”) reflexes can be useful aids in localizing or
diagnosing both central and PNS problems (Fig. 1-3).
FIGURE 1-3. Muscle stretch (“deep tendon”) reflexes. (Reproduced with permission from
Ginsberg L. Lecture Notes: Neurology. 8th ed. Oxford: Blackwell Publishing; 2005:44.)
In the arms, the biceps, brachioradialis, and triceps reflexes are most
commonly tested. A pectoral reflex can be assessed by tapping the pectoralis
muscle and looking for adduction of the proximal arm. Thumb flexion
stimulated by flicking the distal phalanx of the middle finger is a positive
Hoffmann sign, an indication of hyperreflexia. In the legs, patellar (knee jerk)
and ankle reflexes are commonly tested. The adductor reflex can also be
tested by striking the medial thigh and looking for thigh adduction. The
Babinski sign is sought by stroking the lateral sole of the foot while
observing for extension of the great toe. Clonus, if present, can be elicited by
forcibly dorsiflexing the ankle when it is relaxed. In some cases, an
exaggerated jaw jerk can localize a problem above the level of the cervical
spine.
SENSORY EXAM
The sensory examination assesses small-fiber (pinprick, temperature) and
large-fiber (vibration, proprioception) function. Pinprick and temperature
information is carried in the spinothalamic tract. Vibration and proprioception
require dorsal column tract integrity. It is generally helpful to start distally
and move proximally when testing each modality because polyneuropathy,
one of the most common causes of sensory abnormality, generally shows up
first in the toes. Nevertheless, it makes sense to test sensory function more
extensively in any affected limb—even if more distal function is normal—to
look for other patterns of abnormality.
Pinprick: Using a sterile instrument (e.g., special pins designed for the
neurologic exam), the examiner starts to prick the toes and gradually
moves up the leg to assess if there is a gradient to sensation. The process
can be repeated starting in the fingers and moving up the arm. If there is
concern for a spinal cord lesion, it is important to perform pinprick along
the length of the torso to identify a “level” where sensation transitions
from abnormal to normal. If the patient reports facial symptoms, the pin
should be used to assess sensation in areas representing each branch of
the trigeminal nerve.
Temperature: Using a similar approach, a cold tuning fork can be used to
assess temperature sensation.
Vibration: After striking the 128-Hz tuning fork, the stem is placed against a
joint, and the duration for which the stimulus is appreciated is recorded.
In general, this great toe is tested first, with the examiner testing
increasingly proximal joints if the distal findings are abnormal.
Proprioception: Proprioception, or joint position sense, is tested in an order
similar to that used for vibration assessment. Usually, the examiner starts
by holding the sides of the great toe and asking the patient to report when
it is being moved upward and downward by a few millimeters.
Light touch is often not useful to test in isolation because it relies on a
combination of pathways. By itself it is unlikely to provide clues to
localization or diagnosis.
KEY POINTS
● Pinprick and temperature information is carried in the spinothalamic pathway, whereas
vibration and joint position sense are relayed in the dorsal columns.
● It is important to use an organized approach (e.g., always moving distal to proximal along a
limb) when testing sensory function.
COORDINATION
Coordination of the limbs and the trunk should be assessed. Finger-to-nose
testing can identify dysmetria (inaccuracy of targeting) or types of tremor in
the arms. Heel-to-shin testing can elicit incoordination in the legs. To test
axial abnormalities, the patient can be asked to sit upright and unsupported,
with the eyes closed.
Rapid alternating movements, rhythmic finger tapping, and heel tapping
are particularly sensitive to coordination problems. In some disorders, such as
Parkinson’s disease, there can be a hesitation, decremental slowing (i.e.,
damping), or increasingly small excursions with repetitive movements.
Patients may also have trouble with the timing, or cadence, of these
movements. Dysdiadochokinesis is the term used to describe difficulty with
rapid alternating movements.
GAIT
Ambulation is one of the most important elements of the neurologic
examination. Normal gait requires the proper functioning of many different
aspects of the nervous system, so it is one of the most sensitive ways to detect
an abnormality. Furthermore, some patterns of gait abnormality herald the
presence of specific disorders (e.g., parkinsonism). Routinely, posture, base,
initiation, stride length, turning, arm swing, and overall balance are
considered.
Posture should be upright. The patient with a normal base, or stance,
maintains the feet at about hip-width apart. In general, healthy individuals
start walking without any hesitation. Stride length should be full, with
clearance of the feet from the floor. Short-stepped and shuffling gaits are
characterized by decreased stride length and limited excursion of the feet
from the ground. The arms normally swing fully in the opposite direction
from their respective legs during ambulation. Decreased arm swing is often a
feature of extrapyramidal disorders. A normal turn can be executed in two
steps; patients with Parkinson’s disease may take multiple small steps to turn
“en bloc.”
Ataxia of gait results in an inability to walk in a straight line; patients may
stagger from one side to the other or list consistently toward one side. Ataxia
is typically associated with a wide-based stance. Ataxia can be brought out
most obviously by having the patient attempt to walk heel to toe (tandem). A
Romberg sign is present when the patient maintains a steady stance with feet
together and eyes open but sways and falls with feet together and eyes closed;
its presence usually implies a deficit of joint position sense, not cerebellar
dysfunction.
KEY POINTS
● Gait is one of the most important elements of the neurologic exam because it is sensitive for
many deficits, and certain diseases have characteristic gait disorders.
● Base, initiation, stride length, turning, arm swing, and the ability to perform tandem gait
should be assessed.
● A positive Romberg sign suggests a deficit in joint position sense.
2 Neurologic Investigations
TECHNIQUE
Optimal positioning is the key to a successful and atraumatic LP. LP is best
performed with the patient in the lateral decubitus position with the legs
flexed up over the abdomen. Ideally, a pillow should be placed between the
legs, and the patient should lie on the edge of the bed where there is better
support to keep the back straight. The anterosuperior iliac spine is at the level
of the L3–L4 vertebral interspace. The LP may be performed at this level,
one interspace higher, or one to two interspaces lower. (Remember that the
spinal cord ends at the level of L1–L2.) The needle is inserted with the bevel
facing upward, so that it will enter parallel to the ligaments and dura that it
pierces rather than cutting them transversely. The needle is directed slightly
rostrally to coincide with the downward angulation of the spinous processes.
The needle is advanced gently until CSF is obtained. To measure the opening
pressure reliably, the patient’s legs should be extended slightly and note
should be made of fluctuation of the CSF meniscus within the manometer
with respiration.
INTERPRETATION OF RESULTS
CSF is a clear, colorless fluid. The glucose content is about two-thirds that of
blood, and it contains up to 40 to 50 mg/dL protein. Fewer than five cells are
present, and these are lymphocytes. The opening pressure measured by LP in
the lateral recumbent position is about 60 to 150 mm H2O.
Xanthochromia refers to the yellow discoloration of the supernatant of a
spun CSF sample. Its presence helps to distinguish an in vivo intrathecal
hemorrhage from a traumatic tap (in which red blood cells [RBCs] have not
lysed, so the supernatant is still colorless).
The implications of various CSF findings are summarized in Table 2-1.
CSF findings in a variety of common conditions are summarized in Table 2-
2. Special tests may be performed as indicated. Some examples include
cytology for suspected malignancy, oligoclonal banding for suspected
immune-mediated processes such as multiple sclerosis, 14-3-3 protein for
Creutzfeldt–Jakob disease, and a variety of polymerase chain reactions and
serologic tests to detect infections of the nervous system.
KEY POINTS
● A CT scan should be performed prior to LP, especially when there is concern about
increased intracranial pressure or focal neurologic abnormalities.
● LP is performed at or below the L2–L3 interspace.
● Xanthochromia indicates recent intrathecal hemorrhage.
I see increasing reason to believe that the view formed some time
back as to the origin of the Makonde bush is the correct one. I have
no doubt that it is not a natural product, but the result of human
occupation. Those parts of the high country where man—as a very
slight amount of practice enables the eye to perceive at once—has not
yet penetrated with axe and hoe, are still occupied by a splendid
timber forest quite able to sustain a comparison with our mixed
forests in Germany. But wherever man has once built his hut or tilled
his field, this horrible bush springs up. Every phase of this process
may be seen in the course of a couple of hours’ walk along the main
road. From the bush to right or left, one hears the sound of the axe—
not from one spot only, but from several directions at once. A few
steps further on, we can see what is taking place. The brush has been
cut down and piled up in heaps to the height of a yard or more,
between which the trunks of the large trees stand up like the last
pillars of a magnificent ruined building. These, too, present a
melancholy spectacle: the destructive Makonde have ringed them—
cut a broad strip of bark all round to ensure their dying off—and also
piled up pyramids of brush round them. Father and son, mother and
son-in-law, are chopping away perseveringly in the background—too
busy, almost, to look round at the white stranger, who usually excites
so much interest. If you pass by the same place a week later, the piles
of brushwood have disappeared and a thick layer of ashes has taken
the place of the green forest. The large trees stretch their
smouldering trunks and branches in dumb accusation to heaven—if
they have not already fallen and been more or less reduced to ashes,
perhaps only showing as a white stripe on the dark ground.
This work of destruction is carried out by the Makonde alike on the
virgin forest and on the bush which has sprung up on sites already
cultivated and deserted. In the second case they are saved the trouble
of burning the large trees, these being entirely absent in the
secondary bush.
After burning this piece of forest ground and loosening it with the
hoe, the native sows his corn and plants his vegetables. All over the
country, he goes in for bed-culture, which requires, and, in fact,
receives, the most careful attention. Weeds are nowhere tolerated in
the south of German East Africa. The crops may fail on the plains,
where droughts are frequent, but never on the plateau with its
abundant rains and heavy dews. Its fortunate inhabitants even have
the satisfaction of seeing the proud Wayao and Wamakua working
for them as labourers, driven by hunger to serve where they were
accustomed to rule.
But the light, sandy soil is soon exhausted, and would yield no
harvest the second year if cultivated twice running. This fact has
been familiar to the native for ages; consequently he provides in
time, and, while his crop is growing, prepares the next plot with axe
and firebrand. Next year he plants this with his various crops and
lets the first piece lie fallow. For a short time it remains waste and
desolate; then nature steps in to repair the destruction wrought by
man; a thousand new growths spring out of the exhausted soil, and
even the old stumps put forth fresh shoots. Next year the new growth
is up to one’s knees, and in a few years more it is that terrible,
impenetrable bush, which maintains its position till the black
occupier of the land has made the round of all the available sites and
come back to his starting point.
The Makonde are, body and soul, so to speak, one with this bush.
According to my Yao informants, indeed, their name means nothing
else but “bush people.” Their own tradition says that they have been
settled up here for a very long time, but to my surprise they laid great
stress on an original immigration. Their old homes were in the
south-east, near Mikindani and the mouth of the Rovuma, whence
their peaceful forefathers were driven by the continual raids of the
Sakalavas from Madagascar and the warlike Shirazis[47] of the coast,
to take refuge on the almost inaccessible plateau. I have studied
African ethnology for twenty years, but the fact that changes of
population in this apparently quiet and peaceable corner of the earth
could have been occasioned by outside enterprises taking place on
the high seas, was completely new to me. It is, no doubt, however,
correct.
The charming tribal legend of the Makonde—besides informing us
of other interesting matters—explains why they have to live in the
thickest of the bush and a long way from the edge of the plateau,
instead of making their permanent homes beside the purling brooks
and springs of the low country.
“The place where the tribe originated is Mahuta, on the southern
side of the plateau towards the Rovuma, where of old time there was
nothing but thick bush. Out of this bush came a man who never
washed himself or shaved his head, and who ate and drank but little.
He went out and made a human figure from the wood of a tree
growing in the open country, which he took home to his abode in the
bush and there set it upright. In the night this image came to life and
was a woman. The man and woman went down together to the
Rovuma to wash themselves. Here the woman gave birth to a still-
born child. They left that place and passed over the high land into the
valley of the Mbemkuru, where the woman had another child, which
was also born dead. Then they returned to the high bush country of
Mahuta, where the third child was born, which lived and grew up. In
course of time, the couple had many more children, and called
themselves Wamatanda. These were the ancestral stock of the
Makonde, also called Wamakonde,[48] i.e., aborigines. Their
forefather, the man from the bush, gave his children the command to
bury their dead upright, in memory of the mother of their race who
was cut out of wood and awoke to life when standing upright. He also
warned them against settling in the valleys and near large streams,
for sickness and death dwelt there. They were to make it a rule to
have their huts at least an hour’s walk from the nearest watering-
place; then their children would thrive and escape illness.”
The explanation of the name Makonde given by my informants is
somewhat different from that contained in the above legend, which I
extract from a little book (small, but packed with information), by
Pater Adams, entitled Lindi und sein Hinterland. Otherwise, my
results agree exactly with the statements of the legend. Washing?
Hapana—there is no such thing. Why should they do so? As it is, the
supply of water scarcely suffices for cooking and drinking; other
people do not wash, so why should the Makonde distinguish himself
by such needless eccentricity? As for shaving the head, the short,
woolly crop scarcely needs it,[49] so the second ancestral precept is
likewise easy enough to follow. Beyond this, however, there is
nothing ridiculous in the ancestor’s advice. I have obtained from
various local artists a fairly large number of figures carved in wood,
ranging from fifteen to twenty-three inches in height, and
representing women belonging to the great group of the Mavia,
Makonde, and Matambwe tribes. The carving is remarkably well
done and renders the female type with great accuracy, especially the
keloid ornamentation, to be described later on. As to the object and
meaning of their works the sculptors either could or (more probably)
would tell me nothing, and I was forced to content myself with the
scanty information vouchsafed by one man, who said that the figures
were merely intended to represent the nembo—the artificial
deformations of pelele, ear-discs, and keloids. The legend recorded
by Pater Adams places these figures in a new light. They must surely
be more than mere dolls; and we may even venture to assume that
they are—though the majority of present-day Makonde are probably
unaware of the fact—representations of the tribal ancestress.
The references in the legend to the descent from Mahuta to the
Rovuma, and to a journey across the highlands into the Mbekuru
valley, undoubtedly indicate the previous history of the tribe, the
travels of the ancestral pair typifying the migrations of their
descendants. The descent to the neighbouring Rovuma valley, with
its extraordinary fertility and great abundance of game, is intelligible
at a glance—but the crossing of the Lukuledi depression, the ascent
to the Rondo Plateau and the descent to the Mbemkuru, also lie
within the bounds of probability, for all these districts have exactly
the same character as the extreme south. Now, however, comes a
point of especial interest for our bacteriological age. The primitive
Makonde did not enjoy their lives in the marshy river-valleys.
Disease raged among them, and many died. It was only after they
had returned to their original home near Mahuta, that the health
conditions of these people improved. We are very apt to think of the
African as a stupid person whose ignorance of nature is only equalled
by his fear of it, and who looks on all mishaps as caused by evil
spirits and malignant natural powers. It is much more correct to
assume in this case that the people very early learnt to distinguish
districts infested with malaria from those where it is absent.
This knowledge is crystallized in the
ancestral warning against settling in the
valleys and near the great waters, the
dwelling-places of disease and death. At the
same time, for security against the hostile
Mavia south of the Rovuma, it was enacted
that every settlement must be not less than a
certain distance from the southern edge of the
plateau. Such in fact is their mode of life at the
present day. It is not such a bad one, and
certainly they are both safer and more
comfortable than the Makua, the recent
intruders from the south, who have made USUAL METHOD OF
good their footing on the western edge of the CLOSING HUT-DOOR
plateau, extending over a fairly wide belt of
country. Neither Makua nor Makonde show in their dwellings
anything of the size and comeliness of the Yao houses in the plain,
especially at Masasi, Chingulungulu and Zuza’s. Jumbe Chauro, a
Makonde hamlet not far from Newala, on the road to Mahuta, is the
most important settlement of the tribe I have yet seen, and has fairly
spacious huts. But how slovenly is their construction compared with
the palatial residences of the elephant-hunters living in the plain.
The roofs are still more untidy than in the general run of huts during
the dry season, the walls show here and there the scanty beginnings
or the lamentable remains of the mud plastering, and the interior is a
veritable dog-kennel; dirt, dust and disorder everywhere. A few huts
only show any attempt at division into rooms, and this consists
merely of very roughly-made bamboo partitions. In one point alone
have I noticed any indication of progress—in the method of fastening
the door. Houses all over the south are secured in a simple but
ingenious manner. The door consists of a set of stout pieces of wood
or bamboo, tied with bark-string to two cross-pieces, and moving in
two grooves round one of the door-posts, so as to open inwards. If
the owner wishes to leave home, he takes two logs as thick as a man’s
upper arm and about a yard long. One of these is placed obliquely
against the middle of the door from the inside, so as to form an angle
of from 60° to 75° with the ground. He then places the second piece
horizontally across the first, pressing it downward with all his might.
It is kept in place by two strong posts planted in the ground a few
inches inside the door. This fastening is absolutely safe, but of course
cannot be applied to both doors at once, otherwise how could the
owner leave or enter his house? I have not yet succeeded in finding
out how the back door is fastened.