Gait and Station

When proprioception is disturbed, the patient may be
able to stand with eyes open, but sways or falls with eyes closed
(Romberg or Brauch-Romberg sign). The Romberg sign is often
misunderstood and misinterpreted. The essential finding is a difference
between standing balance with eyes open and closed. In order to test
this function, the patient must have a stable stance eyes open and then
demonstrate a decrease in balance with eyes closed, when visual input
is eliminated and the patient must rely on proprioception to maintain
balance.

The Romberg test can be difficult to interpret. There is
some variability, even among expert examiners, in how the Romberg test
is performed and interpreted. Many patients sway slightly with eyes
closed, and minimal amounts of sway, especially in elderly patients,
are seldom significant. Minor, normal swaying may stop if the patient
is simply asked to stand perfectly still. Most clinicians discount sway
at the hips, and insist on seeing sway at the ankles before calling the
test positive; some require the patient take a corrective step to the
side; and some that the patient nearly fall. Some require the patient
be barefoot. The “sharpened” or tandem Romberg is done by having the
patient stand in tandem position with eyes open and closed; the limits
of normality for this variation are conjectural.

The Romberg sign is used primarily as a test of
proprioceptive, not cerebellar, function. The pioneering
nineteenth-century clinicians thought it was particularly useful in
separating tabes dorsalis from cerebellar disease. In fact, patients
with cerebellar disease, particularly disorders of the
vestibulocerebellum or spinocerebellum, may have some increase in
instability with eyes closed, but not usually to the degree seen with
impaired proprioception. A patient with an acute unilateral
vestibulopathy may fall toward the side of the lesion when standing
with eyes closed. Patients with cerebellar disease, or those with
severe weakness, may not have a stable base eyes open. It may help to
have the patient widen stance to the point where they are stable eyes
open, then close the eyes, and check for any difference. Only a marked
worsening of balance with eyes closed qualifies as a positive Romberg
sign. A patient who cannot maintain balance feet together and eyes open
does not have a positive Romberg.

Some histrionic patients will sway with eyes closed in
the absence of any organic neurologic impairment (false Romberg sign).
The swaying is usually from the hips and may be exaggerated. If the
patient takes a step, the eyes may remain closed, which never happens
with a bona fide Romberg. The instability can often be eliminated by
diverting the patient’s attention. Effective distractors are to ask the
patient to detect numbers the examiner writes with her finger on the
forehead, wiggle the tongue, or to perform finger-to-nose testing.
Having the shoes off and watching the toe movements may be very
informative. The toes of the patient with histrionic sway are often
extended; the patient with organic imbalance flexes the toes strongly
and tries to grip the floor.

The gait of cerebellar disease is caused by involvement
of the coordinating mechanisms in the cerebellum and its connecting
systems. There is a clumsy, staggering, unsteady, irregular, lurching,
titubating, wide-based gait, and the patient may sway to either side,
back, or forward. Leg movements are erratic, and step length varies
unpredictably. The patient is unable to walk tandem or follow a
straight line on the floor. There may be tremors and oscillatory
movements involving the entire body. With a lesion of the cerebellar
vermis, the patient will exhibit a lurching, staggering gait, but
without laterality, the ataxia will be as marked toward one side as the
other. Cerebellar ataxia is present with eyes both open and closed; it
may increase slightly with eyes closed, but not so markedly as in
sensory ataxia. A gait resembling cerebellar ataxia is seen in acute
alcohol intoxication. With a hemispheric lesion the patient will
stagger and deviate toward the involved side. In disease localized to
one cerebellar hemisphere or in unilateral vestibular disease, there is
persistent swaying or deviation toward the abnormal side. As the
patient attempts to walk a straight line or to walk tandem she deviates
toward the side of the lesion. Walking a few steps backward and forward
with eyes closed may bring out “compass deviation” or a “star-shaped
gait.” When attempting to walk a fixed circle around a chair, clockwise
then counterclockwise, the patient will tend to fall toward the chair
if it is on the side of the lesion, or to spiral out away from the
chair if on the opposite side. Either unilateral cerebellar or
vestibular disease may cause turning toward the side of the lesion on
the Fukuda stepping test. For all the tests that bring out deviation in
one direction, other findings must be used to differentiate between
vestibulopathy and a cerebellar hemispheric lesion. Unilateral ataxia
may be demonstrated by having the patient attempt to jump on one foot,
with the eyes either open or closed. The patient with bilateral
vestibular disease may seek to minimize head movement during walking,
holding the head stiff and rigid; having the patient turn the head back
and forth during walking may bring out ataxia.

Sensory ataxia occurs when the nervous system is
deprived of the sensory information, primarily proprioceptive,
necessary to coordinate walking. Deafferentation may result from
disease of the posterior columns (e.g., tabes dorsalis or subacute
combined degeneration), or disease affecting the peripheral nerves
(e.g., sensory peripheral neuropathy). The term spinal ataxia is
sometimes used, but the pathology is not always in the spinal cord. The
patient loses awareness of the position of the lower extremities in
space, or even of the body as a whole, except as provided by the visual
system. The patient is extremely dependent on visual input for
coordination. When deprived of visual input, as with eyes closed or in
the dark, the gait deteriorates markedly. The difference in walking
ability with and without visual input is the key feature of sensory
ataxia. If the condition is mild, locomotion may appear normal when the
patient walks eyes open; more commonly it is wide based, and poorly
coordinated.

The term “steppage gait” refers to a manner of walking
in which the patient takes unusually high steps. Sensory ataxia is one
of the causes of a steppage gait. The patient takes a high step, throws
out her foot, and slams it down on the floor in order to increase the
proprioceptive feedback. The heel may land before the toe, creating an
audible “double tap.” An additional sound effect may be the tapping of
a cane, creating a “slam, slam, tap” cadence. The sound effects may be
so characteristic that the trained observer can make the diagnosis by
listening to the footfalls. The patient with

sensory
ataxia watches her feet and keeps her eyes on the floor while walking.
With eyes closed, the feet seem to shoot out, the staggering and
unsteadiness are increased, and the patient may be unable to walk.
There is less reeling and lurching in sensory ataxia than with a
comparable degree of cerebellar ataxia. The difficulty is even worse
walking backward, since the patient cannot see where she is going. The
patient with bilateral foot drops, however, also has a steppage gait
and a double tapping sound. In all of these tests, sensory ataxia can
be differentiated from predominantly cerebellar ataxia by accentuation
of the difficulty with eyes closed; and unilateral cerebellar or
vestibular disease from vermis involvement by laterality of
unsteadiness.

The gait of spastic hemiparesis may be caused by a
lesion interrupting the corticospinal pathways to one half of the body,
most commonly stroke. The patient stands with a hemiparetic posture,
arm flexed, adducted, and internally rotated, and leg extended (Figure 19.1).
There is plantar flexion of the foot and toes, either due to foot
dorsiflexion weakness or to heel cord shortening, rendering the lower
extremity on the involved side functionally slightly longer than on the
normal side, referred to as an equinus deformity. When walking, the
patient holds the arm tightly to the side, rigid and flexed; she
extends it with difficulty and does not swing it in a normal fashion.
She holds the leg stiffly in extension and flexes it with difficulty.
Consequently, the patient drags or shuffles the foot and scrapes the
toes. With each step, she may tilt the pelvis upward on the involved
side to aid in lifting the toe off the floor (hip hike) and may swing
the entire extremity around in a semicircle from the hip
(circumduction). The stance phase is shortened because of weakness, and
the swing phase shortened because of spasticity and slowing of
movement. The sound produced by the scraping of the toe, as well as the
wear of the shoe at the toe, may be quite characteristic. The patient
is able to turn toward the paralyzed side more easily than toward the
normal side. Loss of normal arm swing and slight circumduction of the
leg may be the only gait abnormalities in very mild hemiparesis.

This gait pattern occurs in patients who have severe
spasticity of the legs. It occurs in patients who have congenital
spastic diplegia (Little disease, cerebral palsy) and related
conditions, and in chronic myelopathies due to conditions such as
multiple sclerosis and cervical spondylosis. There is characteristic
tightness of the hip adductors causing adduction of the thighs, so that
the knees may cross, one in front of the other, with each step
(scissors gait). The patient walks on an abnormally narrow base, with a
stiff shuffling gait, dragging both legs and scraping the toes. The
steps are short and slow; the feet seem to stick to the floor. There
may be a marked compensatory sway of the trunk away from the side of
the advancing leg. Swaying and staggering may suggest an element of
ataxia, but usually there is no true loss of coordination. The
shuffling, scraping sound—together with worn areas at the toes of the
shoes—are characteristic. The equinus position of the feet and heel
cord shortening often cause the patient to walk on tiptoe.

Some neurologic disorders cause involvement of both the
corticospinal and the proprioceptive pathways (e.g., combined system
disease due to vitamin B12 deficiency, or multiple sclerosis),
resulting in a gait that has features of both spasticity and ataxia.
The relative proportion of each abnormality depends on the particulars
of the case. The ataxic component may be either cerebellar or sensory.
In vitamin B12 deficiency it is predominantly sensory; in multiple
sclerosis, both components may be present. In amyotrophic lateral
sclerosis there may be bilateral foot drops, as well as spasticity,
resulting in an abnormality in walking that may suggest a spastic
ataxic gait. The gait has been described as “jiggling” or “bobbing,”
with tremulous, bouncing, up-and-down body movements.

The gait in most akinetic-rigid, parkinsonian syndromes
is characterized by rigidity, bradykinesia, and loss of associated
movements. The patient is stooped, with head and neck forward and knees
flexed; the upper extremities are flexed at the shoulders, elbows, and
wrists, but the fingers are usually extended (Figure 21.1).
The gait is slow, stiff, and shuffling; the patient walks with small,
mincing steps. Difficulty walking may be one of the earliest symptoms
of the disease. The same gait disorder can occur with any condition
causing parkinsonism, such as drug side effects.

The marche à petits pas (walk of little steps) gait
resembles that of parkinsonism, but lacks the rigidity and
bradykinesia. Locomotion is slow, and the patient walks with very
short, mincing, shuffling, and somewhat irregular footsteps. The length
of the step may be less than the length of the foot. There is often a
loss of associated movements. This type of gait may be seen in normal
elderly persons, but also occurs in patients who have diffuse cerebral
hemispheric dysfunction, particularly involving the frontal lobes. It
may also occur as part of the syndrome of normal pressure
hydrocephalus, and in other types of hydrocephalus. The same gait
disturbance is typical of multi-infarct dementia or lacunar state. In
some patients with marche à petits pas there are bizarre movements such
as dancing or hopping. There may be generalized weakness of the lower
extremities or of the entire body, with the patient fatiguing easily.

Apraxia of gait is the loss of the ability to use the
legs properly in walking, without demonstrable sensory impairment,
weakness, incoordination, or other apparent explanation. Gait apraxia
is seen in patients with extensive cerebral lesions, especially of the
frontal lobes. It is a common feature of normal pressure hydrocephalus,
and may occur in frontal lobe neoplasms, Binswanger disease, Pick
disease, and other conditions that cause diffuse frontal lobe
dysfunction. The patient cannot carry out purposeful movements with the
legs and feet, such as making a circle or kicking an imaginary ball. In
rising, standing, and walking there is difficulty in initiating
movement, and the automatic sequence of component movements is lost.
The gait is slow and shuffling, with short steps. The patients may have
the greatest difficulty initiating walking, making small, feeble,
stepping movements with minimal forward progress. Eventually the
patient may be essentially unable to lift the feet from the floor, as
if they were stuck or glued down; or may raise them in place without
advancing them (magnetic gait, gait ignition failure, start
hesitation). After a few hesitant shuffles, the stride length may
increase (slipping clutch gait). When trying to turn, the patient may
freeze (turn hesitation). The patient may be able to imitate normal
walking movements when sitting or lying down, but eventually even this
ability is often lost. In addition, perseveration, hypokinesia,
rigidity, and stiffness of the limb in response to contact
(gegenhalten) are often seen.

The abnormal gait that occurs in frontal lobe disease is
difficult to describe and classify, and is in many ways similar to,
perhaps identical to, gait apraxia. Many terms have been used, which
refer to more or less the same phenomenon, including: gait apraxia,
frontal disequilibrium, Bruns apraxia/ataxia, magnetic gait, and lower
half/body parkinsonism. Lesions of the frontal lobe, or of the frontal
lobe connections with the basal ganglia and cerebellum, may lead to a
gait disorder characterized by a slightly flexed posture, short,
shuffling steps, and an inability to integrate and coordinate
lower-extremity movements to accomplish normal ambulation. The disorder
has been attributed to involvement of the frontopontocerebellar fibers
at their origin in the frontal lobe, but the explanation is probably
more complex. The abnormality is very similar to gait apraxia.

A problem arises with the use of the term “steppage,”
which means that the patient is lifting one or both legs high during
their respective stride phases, as though she were walking up steps
though the surface is level. Patients with foot drop may do this in
order to help the foot clear the floor and avoid tripping. Patients
with sensory ataxia, classically tabes dorsalis, may also lift the feet
up high and then slap them down smartly to improve proprioceptive
feedback. Since both of these gaits are “high-stepping,” both have been
referred to as steppage gaits, but the causes and mechanisms are quite
different.

A patient with foot drop has weakness of the
dorsiflexors of the foot and toes. When mild, this may be manifest only
as a decrease in the toe clearance during the stride phase. With more
severe foot drop the patient is in danger of tripping, and may drag the
toe when she walks, characteristically wearing out the toe of her shoe.
When foot drop is severe, the foot dangles uncontrollably during the
swing phase. To compensate, she lifts the foot as high as possible,
hiking the hip and flexing the hip and knee. The foot is thrown out and
falls to the floor, toe first. The touching of the toe, followed by the
heel creates a “double tap” that has a different sound than the
heel-first double tap of sensory ataxia. The patient is unable to stand
on her heel, and when standing with her foot projecting over the edge
of a step, the forefoot drops. The foot drops and steppage gait may be
unilateral or bilateral. Common causes of unilateral foot drop and
steppage gait include peroneal nerve palsy and L5 radiculopathy. Causes
of bilateral foot drop and steppage gait include amyotrophic lateral
sclerosis, Charcot-Marie-Tooth disease and other severe peripheral
neuropathies, and certain forms of muscular dystrophy.

Myopathic gaits occur when there is weakness of the hip
girdle muscles, most often due to myopathy, and most characteristically
due to muscular dystrophy. If the hip flexors are weak there may be a
pronounced lordosis. The hip abductor muscles are vital in stabilizing
the pelvis while walking. Trendelenburg sign is an abnormal drop of the
pelvis on the side of the swing leg due to hip abductor weakness. When
the weakness is bilateral, there is an exaggerated pelvic swing that
results in a waddling gait. The patient walks with a broad base, with
an exaggerated rotation of the pelvis, rolling or throwing the hips
from side to side with every step to shift the weight of the body. In
the extreme forms, this gait pattern has a bizarre appearance. The
patient walks with a pronounced waddle, shoulders thrown back and
pelvis thrust forward. This form of gait is particularly common in
facioscapulohumeral muscular dystrophy. The myopathy patient has marked
difficulty climbing stairs, often needing to pull herself up with the
hand rail. Patients also have difficulty going from a lying to standing
position without placing the hands on the knees and hips to push
themselves up (Gowers sign, Fig. 20.3).

A cautious gait is seen in older patients who have no
neurologic disease but are uncertain of their balance and postural
reflexes. The gait takes on the characteristics seen when a healthy
person walks on an icy surface: velocity slows, steps shorten, and the
base widens.

In conditions such as Sydenham chorea, Huntington
disease, other forms of transient or persistent chorea, athetosis, and
dystonia the abnormal movements may become more marked while the
patient is walking, and the manifestations of the disease more evident.
Walking may accentuate not only the hyperkinesias, but also the
abnormalities of power and tone that accompany them. In Huntington
disease, the gait may be grotesque, dancing or prancing with abundant
extraneous movement. It may look histrionic but is all too real. In
athetosis, the distal movements, and in dystonia the proximal
movements, may be marked during walking, and in both there are
accompanying grimaces.

In addition to the steppage gait that accompanies foot
drop, weakness limited to other muscle groups may cause gait
difficulties. With paralysis of the gastrocnemius and soleus muscles,
the patient is unable to stand on the toes, and unable to push off to
enter the swing phase with the affected leg. This may cause a shuffling
gait that is devoid of spring. In weakness of the quadriceps muscle
(e.g., femoral neuropathy), there is weakness of knee extension, and
the patient can only accept weight on the affected extremity by bracing
the knee. When walking the knee is held stiffly, and there is a
tendency to fall if the knee bends. The patient has less difficulty
walking backward than forward. Lumbosacral radiculopathy may cause
either foot drop or a unilateral Trendelenberg gait, or both. In
addition, the patient with acute radiculopathy may walk with a list or
pelvic tilt, accompanied by flattening of the normal lumbar lordosis
because of low back muscle spasm. The patient may walk with small
steps; if the pain is severe, she may place only the toes on the floor,
since dorsiflexion of the foot aggravates the pain. Patients commonly
use a cane to avoid bearing weight on the involved leg.