Seminars in
ARTHRITIS AND RHEUMATISM AUGUST
VOL 20, NO 1
Anatomy
and Physiology
of the Cervical
By John H. Bland and Dallas Although
the
studied spine
lumbar
from
1934
has received
physiological,
of the are
authors
spines
and
logically, article
collected
studied
them
and
nucleus
nerve
root
(anterior) volume
to
anatomy
spinal canal,
size
menisci
and the anatomy autonomic
0 1990 by W.B. INDEX
WORDS:
physiology:
in this
anatomic
charac-
the
sites,
position
uncinate
relation
shape
anterior
system
of the
of spinal
of the and
spinal
posterior
of the zygapophyseal
and clinical
nervous
human physio-
pulposus,
and
of the
The
in 1955,
whole
Reported
root,
often
warrant
to
anatomically,
exit
nerve
are spine.
Beginning 171
important
of the
cord
spine
histologically.
are clinically
Anatomic,
cervical
correlation.
cervical
biomechanical
extensive
have
process,
canal,
lumbar
too
teristics motor
and
to the
far
such an assumed the
extensively the
far less attention.
to apply
differences
was
present,
biochemical,
characteristics presumed
spine
to the
significance
in the cervical
joints, of the spine.
Saunders Company. Neck:
cervical
spine;
anatomy:
pathology.
C
LINICAL SYMPTOMS and signs usually reflect abnormal function of a tissue or organ system. Disease in most instances has a visible component and the study of morbid anatomy was, and should now be, the classic approach to its understanding. Recently, there has been considerable interest in molecular and cell biology; gross anatomic studies are regarded as outmoded, old fashioned-even irrelevant! Still, as William Blake wrote, one need not be limited to the “Vegetated Mortal Eye’s perverted and single, flat vision” if one looks not with, but through, the eye.*?
*Blake W: Jerusalem, in Keynes G (ed): The Writings of William Blake (vol 3). London, England, Nonesuch Press, 1925, Ch 3, line 11. *Buliough PC: Understanding osteoarthritis: The value of anatomic studies. J Rheumatoi 14:189-190,1987
1990
Spine
R. Boushey
The major medical advances over the last 100 years stem from an intensive study of biochemistry and immunology; evolution and maturation of cell biology illuminated morbid anatomy, but only because morbid anatomy was studied in great detail, eg, immunofluorescent studies in renal pathology allowed us to see “through the eye,” disclosing the detailed cellular and molecular understanding of these diseases. Although it is too little appreciated, morbid anatomy remains a key to understanding clinical disease. In the case of the cervical spine, there has been relatively little morbid and microscopic anatomy, presumably because of difficulty of obtaining whole human cervical spine specimen. This article reflects our deep, enduring interest in cervical spine anatomy dating to a single clinical experience in 1955 when the first spine studied was bequeathed to one of the authors (JHB). Over this period, a total of 17 1 whole human cervical spines have been removed post mortem, obtained from anatomical laboratories and the coroner’s office.
From the University of Vermont College of Medicine, Department of Medicine, the Rheumatology and Clinical Immunology Unit, and the Department of Anatomy and Neurobiology, Section of Gross Anatomy, Burlington. John H. Bland, MD: Professor Emeritus of MedicineRheumatology. Department of Medicine. and the Rheumatology and Clinical Immunology Unit, University of Vermont, Burlington; Dallas R. Boushey, Assistant Professor Emeritus of Anatomy and Neurobiology, the Department of Anatomy and Neurobiology, Section of Gross Anatomy. Supported by Research Grants from the National Institutes of Health, Arthritis and Rheumatism Branch and by the Irene Heinz Given and John LaPorte Given Foundation. Address reprint requests to John H. Bland, MD. University of Vermont College of Medicine, Rheumatology and Clinical Immunology Unit, Given Medical Bldg. Rm 0302, Burlington, VT 05405. Q 1990 by W.B. Saunders Company. 0049-0172,f90/2001-OOOl$5.00/0
Seminars in Arthritisand Rheumatism, Vol 20, No 1 (August). 1990: pp l-20
2
BLAND AND BOUSHEY
The cervical spine is the most complicated articular system in the body; there are 37 separate joints that function to carry out the myriad movements of the head and neck in relation to the trunk and subserve all specialized sense organs, eg, eyes, ears, nose, and tongue. The seven small cervical vertebrae with their ligamentous, capsular, tendinous, and muscle attachments are poorly designed to protect their contents, compared with the skull above and the thorax below. The contents of this anatomical cylinder interposed between skull and thorax include carotid and vertebral arteries, the spinal cord, the anterior and posterior nerve roots, and in its uppermost portion, the brain stem. The extremely flexible cervical spine balances a 10 to 15 pound ball, the head, on the lateral masses (zygapophyseal joints) of the atlas. The head acts as a cantilever on top of the highly mobile neck. Normally the neck moves over 600 times an hour, whether we’re awake or asleep; no other part of the musculoskeletal system is in such constant motion. The cervical spine is subject to stress and strain in ordinary, every day activities, such as speaking, gesturing, rising, sitting, walking, turning, and even at rest lying down. The position of the cervical spine discloses mood and attitude. The spine in flexion suggests depression, withdrawal, sadness, mourning, and sometimes prayer, (“bowing” the head in supplication); while “chin extension of the cervical spine, reflects up,” optimism, confidence, and savoir faire. A “pain in the neck” is such an unpleasant experience that it is used as an invective for any annoying event, even a person. The word “neck” is also used as a verb-to neck-as in kissing and caressing in love making, currently out of style! Normal function requires that all movements be made without damaging the spinal cord, vascular supply, and millions of nerve fibers.
CLINICAL SIGNIFICANCE
Is the cervical spine clinically important? In one epidemiological study, over 10% of the population recalled having had at least three episodes of pain in the neck last year. Another reports that at any one specific time as much as 12% of the women and 9% of the men in the population experience neck pain with or without associated arm pain, and 35% of people can recall such an
episode.2 An important clinical epidemiological study disclosed that a history of stiff neck and arm pain was elicited in 80% of a population of male industrial and forest workers. In a second study by the same investigators, the figure was modified to 5 1% in a series of 1,193 male workers (a broad spectrum of jobs) but only 5% experienced work loss as a consequence of the pain.3*4 Further study of neck pain reports that 70% of adults who visited their doctors were well or improving within 1 month.’ There is little else known about the epidemiology of acute or chronic neck symptoms. From general clinical experience and these scant insights, we postulate that neck pain is a recurring ubiquitous clinical event, a mild to modest transitory nuisance to most of us. However, with such enormous morbidity, if even a small percentage of those with symptoms seek medical diagnosis and care, the impact on society would be striking and dramatic. We strongly suspect that such is the case, but the relevant epidemiological data are concealed in such clinical headings as disc disease, spine injuries, and injuries to the trunk where the cervical pain syndromes are overwhelmed by low back pain. With so much morbidity, there is precious little systematic information on the clinical features and natural history of neck disease. Until 1948, shoulder, scapular, chest, arm, and forearm pain syndromes were mysteries, That year Lord Russell Brain6 described a myelopathy-spinal cord compressionthat was clearly, unequivocally ascribed to primary cervical spine disc disease, promptly termed cervical spondylosis. The concept was accepted, and a whole spectrum of mysterious clinical syndromes became clearly understood.
A HISTORICAL VIGNETTE
There is an important relation between backache and neck pain. During and after World War I, backache was a rarely reported problem; but when World War II began, backache was the diagnosis in 40% of patients admitted to a general military hospital with “arthritis and allied conditions.“7 The period between wars saw backache emerge as a major medical problem for the military, industrial medicine, the insurance industry, public health, and certainly medicine in
ANATOMY
AND PHYSIOLOGY OF THE CERVICAL SPINE
general.’ Why the emergence of backache in the 193Os? The expression “my aching back” arose but was soon superseded by “my injured back.” The notion of injury implies trauma, damage, and somebody or something is at fault, ie, culpability. This general concept of injury emerged out of the coincidence of two events: first, an astute clinical observation made at the University of Vermont; second, the introduction of the Worker’s Compensation program. The clinical observation was made over 50 years ago by R.M.P. Donaghy, a neurosurgeon, and Albert Mackay, a general surgeon.g The patient was a 25year-old man injured in a twisting ski accident. The doctors suspected and formulated the idea of a herniated nucleus pulposus; such had not been described then. The patient was referred to Dr Frank Ober in Boston and seen by his associate, Dr Barr who referred him to Dr W. Jason Mixter at the Massachusetts General Hospital in Boston. Multiple sclerosis was a considered diagnosis, and following myelogram, a probable diagnosis of tumor of the high cauda equina was made and exploration followed. An “enchondroma” (nucleus pulposus) was removed, and the patient was cured (June 1932).” In 1934, Mixter and Barr attributed backache in 19 patients to herniation of the nucleus pulposus, demonstrated surgical cure, and named the condition “rupture of the intervertebral disc.“” At the time all state legislatures and judiciaries were considering Workman’s Compensation statutes. Subsequently, all states passed laws that provide medical care and compensation for wages lost due to “personal injury that arises out of and in the course of employment and occurs by accident.” Lawyers think of “rupture” as a rip, tear, or bursting of normal anatomical structure, thus it was a “personal injury.” Even in 1989 a worker with a backache given a diagnosis of “ruptured disc” will be compensated irrespective of a defined discrete cause of their symptoms. A surgical scar suppresses arguments to the contrary much more reliably than the surgical procedure results in cure. From 1934 to about 1965, both clinicians and the courts thought that “ruptured disc” was the cause of most persistent and disabling backache. These views changed gradually as the confidence
3
of diagnosticians, enthusiasm of surgeons, and self-righteousness of the compensation bureaucracy has faded. Epidemiological studies belied those views. The cause of most regional backaches today is indeterminate.“,” Ruptured disc is common in asymptomatic patients. “Backache” is no longer a surgical disease. Greater than 80% of such patients will be well or much better in 2 weeks, with most of the remainder recovering, provided they are well cared for. In this same era, valid and sophisticated research on the lumbar spine was accomplished, as was clinical, radiological, anatomic, pathological, biomechanical, biochemical, physiochemical, and histological research. This was an enormous advance in knowledge of the lumbar spine, but not the cervical spine. There has been very little penetrating research done on the cervical spine. In fact, even the basic anatomy is incompletely explored. During this historical period, the anatomic, radiological, clinical, biomechanical, and biochemical characteristics of the lumbar spine were transferred in the minds of physicians as applicable to the cervical spine. Too many gross differences between the lumbar and cervical spine allow this extrapolation. There are obvious differences. (1) The neck is designed for relatively enormous degrees of mobility. (2) Vertebrae are much smaller and far more anatomically complicated. (3) Zygapophyseal joints are oriented on very different planes. (4) Discs are grossly different-physiologically, anatomically, biomechanically, biochemically, and embryologically. (5) After age 45, there is little or no nucleus pulposus. (6) The lumbar spine is designed for weight bearing and serves a totally different biomechanical function. The lumbar spine is hydrodynamically different than the cervical spine. (7) Cervical discs are far more ligamentous and dry, with little proteoglycan present. (8) The forces operating in the neck are different, not bearing the whole body weight but being influenced by the great mobility of the upper extremities. (9) The pain patterns arising from sclerotomeand myotome-derived structures in the neck are very distinctive and perhaps more clinically usable than those of the lumbar spine. (10) Myelopathy is much more common in the cervical spine than radiculopathy. (11) The diagnostic use of roentgenogram, myelogram,
4
BLAND AND BOUSHEY
computed tomography (CT) scan, and magnetic resonance imaging is less meaningful in the cervical spine than in the lumbar spine. SOME PHYLOGENETIC MATTERS OF CLINICAL IMPORTANCE 1. The occipital bone and the posterior fossa have evolved from the reptilian upper four vertebrae. The nerve supply to this area is from Cl, 2, and 3, and clinical disease in that area is reflected in the referral patterns of those roots; myotomaland sclerotomal-derived structures, when abnormal, are perceived in the forehead and retroorbital areas. 2. The transverse processes in the cervical spine have evolved into the intervertebral foramina and the gargoyle-like structures that carry the anterior and posterior nerve roots and the posterior root ganglion. The nerves occupy about 25% of the space available. The remainder of the space is occupied by lymphatics, blood vessels, areolar and fatty tissue, constituting a compressible safety cushion allowing physiologic encroachment without damage to nerve roots. The bony prominence, the uncus or uncinate process, on the lateral and posterolateral aspect of the cervical vertebrae from C3 through C7 constitutes the phylogenetic remainder of the costovertebral joint in reptiles. Of clinical significance, this bony elevation enlarges from age 9 to 14 years, and beyond the age of 40 years it constitutes a bony bulwark preventing herniation laterally or posterolaterally. 3. The odontoid evolved from the body of the first cervical vertebra that became the atlas. Thus, free movement of the head became possible with the eccentrically located “axle” of the axis, the odontoid, allowing enormous mobility at that level, especially in rotation. 4. With the evolution of the extremities, the neural traffic increased at the level of the spine where extremities appeared. The spinal cord enlargements reflect this evolution. The nerve roots (particularly the sensory roots) are much larger in the cervical spine than in the lumbar and thoracic spine. Riblessness in the cervical and lumbar areas had great survival value because it allowed growth of limbs and their associated neural innervation. 5. The nucleus pulposus, present at birth, and
remaining until age 9 to 14 years, is made of remnants of primitive notochord and proteoglycan material. However, in the cervical spine, contrary to events in the lumbar spine, the nucleus pulposus gradually disappears. In 17 1 whole human cervical spines, we found no evidence of a gel-like nucleus pulposus after age 45. The intervertebral disc in the cervical spine is “dry,” more like a ligament than a “disc,” fibrous, and gradually breaking up in various sized pieces, seemingly a universal, probably physiological, development. CLINICAL SYNDROMES IN THE CERVICAL SPINE
In medical practice, there is an expectation that by detecting a spectrum of clinical signs and symptoms, a definitive diagnosis can be made and treatment instituted. The flaw in this expectation is that cervical spine complaints have been classified into various empirically defined syndromes, each with an alleged basis. The implication is that if one identifies the features said to be characteristic of each syndrome, a diagnosis can be made. In the case of the cervical spine, there has never been a clinical-pathological correlation. The field of cervical spine pain is dominated by descriptions of syndromes offered by various “authorities” as if their underlying pathophysiology had been determined. It has not. Authorities view similar complaints in greatly different ways. Some view neck pain as entirely discal in origin, others prefer the zygapophyseal joints, muscle imbalance, poor posture, and psychogenic origins. The most common complaint related to the cervical spine practice is pain-its localization, and origin. There is no point in proceeding to evaluate therapy if the pain-sensitive structure is not known. Traditional techniques of clinical investigation-roentgenograms, myelography, CT scans, magnetic resonance imaging-do not show sources of pain. Clinical pathological correlations have not been established to prove that a particular radiological abnormality is diagnostic of the source of pain. In fact, there is no correiation between radiological manifestations and clinical symptoms and signs. Except for studies by Tondury,13 Payne and Spillane, I4 Holt and Yates, I5 Frykholm,16 and
ANATOMY
5
AND PHYSIOLOGY OF THE CERVICAL SPINE
removed and studied 17 1 whole human cervical spines. This article reports our anatomic findings. METHODS The cervical spine was removed intact by sawing through the first thoracic vertebra, cutting the occipital membrane ligaments, connecting the posterior arch of the atlas to the occipital bone, and then, with a sharp chisel, cutting off of the occipital condyles. Each specimen included the atlantooccipital joint intact. The whole cervical spine, by blunt and sharp dissection, was complete with spinal cord, all muscles, tendons, ligaments, vessels, and joints to the middle of the first thoracic vertebra. The foramen magnum was removed from some of the later cadavers. Viscera and brain are removed in the routine manner, and the cadaver is placed in a supine position with a block under the neck. A chisel incision is made which encircles the foramen magnum from the inner side of the skull and passes posteriorly through the squamous occipital bone. The incision is marked lightly and deep incision is carried forward through the petrous temporal and basioccipital bones. Cracking the squamous occipital bone is thus avoided.14 The whole cervical spine was photographed in color anteriorly, posteriorly, and laterally (Figs 1 and 2). Roentgenograms are taken antero-posteriorly (AP), laterally, and both oblique views. The necks were fixed in 10% formalin for 14 days, after which they were cut with a thin blade band saw into sagittal, coronal, and transverse slabs of up to 10 mm thickness (Fig 3). We found that we learned the most from the sagittal slabs, the majority were so cut. Roentgenograms were taken of all the slabs, and arranged in order from one Fig
1:
spine,
Specimen vertically
posterior shows tate
aspect. the
of a whole positioned Upper
one third
cord
suspended
spinal
ligaments
(arrowheads);
canal at the foramen
magnum
arrow);
condyles
the occipital
skull with the
edges
visible arches
a sharp of
human and
the
(arrows): of the atlas
chisel
by the
capacious level (short, were
(laterally
anterior
from
of the picture denspinal open
cut off the
situated)
and
joint
are
atlanto-occipital the
cervical
viewed
and
posterior
can be identified.
Hirsch et al,” there have been relatively few detailed gross and microscopic anatomic and pathological studies on the human cervical spine. This investigation was undertaken with the belief that clinical, radiological, post mortem anatomic, and pathological studies would provide a necessary basic background knowledge for understanding and rationally managing clinical disorders of the cervical spine, mainly cervical osteoarthritis. Between 1954 and 1984 we have
Fig 2:
A close-up
odontoid
atlanto-occipital (arrows), visible.
view
“peeking”
joints
the There
surrounding
of Figure
through
hyaline
are more cartilage
is gross
the odontoid.
1 showing
(arrowhead); apparent surfaces
granulomatous
the the here being
tissue
6
BLAND AND BOUSHEY
side to the other (Fig 4). Figures 5, 6, and 7 illustrate respectively a sagittal section of a whole spine, a histological section of the whole spine, including the meninges and spinal cord, and a histological sagittal section of the axis and atlas. All gross specimens were examined simultaneously by an anatomist and a rheumatologist using a dissecting microscope with magnification up to 40x. Normal and abnormal anatomical observations were made. The transverse and AP diameter of the spinal canal was measured in millimeters. Anterior and posterior vertebral osteophytes were identified
Fig 4:
This illustration
shows the radiological
study in serial array, sagittal slabs in Figure 3, permitting microscopic
This illustration
Fig 3:
nique of obtaining
demonstrates
the tech-
sagittal sections of a whole
cervical spine, cut with a thin blade band saw from
the left through
the midline to the right
side. In this specimen
12 sagittal sections were
cut. Sections
for histological
hematoxylin
eosin staining allow a serial three
and
dimensional
build up of any desired set of microscopic tissue planes,
eg. from
intervertebral
the spinal canal through
foramina.
correlation
of roentgenograms
with
histological data.
and graded as 0, 1, 2, or 3.* Gross pathological criteria for osteoarthritis were agreed upon and the zygapophyseal joints, right and left, were graded 0, 1,2, or 3.* The ligamenta flava were assessed for hypertrophy and graded 0, 1, 2, or 3.* Intervertebral discs were graded 0, 1, 2, or 3’ depending on the extent of fissures identified and their connection with the so called joints of Luschka. Decalcification was accomplished by immersing the necks in 10% formic acid, testing for calcium every few days, and changing the solution at intervals for 10 to 16 weeks. When the test for calcium became negative the spine was removed and prepared for sectioning and histological study. In many instances whole spines were cut, serially stained with hematoxylin and eosin, and examined. Smaller sections were cut to study dural root sleeve fibrosis, lesions of the nerve roots, intervertebral foraminal lesions or specific disc joints, and zygapophyseal or Luschka joints. Individual specimens or parts of specimens were cut in one of four planes, sagittal, coronal, oblique, or transverse, depending on the study made and question asked. Sagittal sections show the degree of lordosis, shape and size of vertebral bodies, intervertebral discs, and articular columns with the zygapophyseal joints. Coronal sections best show the shape of vertebral bodies,
the
*Graded: 0, absent; 1, definite; 2, moderate; 3, severe.
ANATOMY
AND PHYSIOLOGY OF THE CERVICAL SPINE
ANATOMIC
STUDIES
Pulposus
Nucleus
The nuclei pulposi of the cervical intervertebral discs, present at birth, are progressively less evident in adolescence, and by age 40 years have disappeared. The adult disc is ligamentous-like, “dry,” and is composed of fibrocartilage, islands of hyaline cartilage, and tendon-like material,
This
Fig 5: section
specimen
of a cervical
is a gross
spine.
the atlas
at the top (arrow
is eroded
and the anterior
is filled spinal pressed
with cord
is cut
normal
matoid
very
plane
and narrow
did
C5-6,
joint
(arrow)
the
and is com-
intervertebral
but
slab
arch of
the odontoid
tissue;
level; the C2-3
height
microscopically discs were
head);
in sagittal
by extensive
midline
anterior
atlanto-occipital
granulomatous
loma at the C5-6 are
Note
disc granuand C3-4
contain C6-7,
discs
granuloma and
and destroyed
C7-Tl by rheu-
granuloma. Fig 6:
This illustration
of a whole intervertebral discs, articular columns with zygapophyseal joints, and the “Luschka joints” (uncovertebral, neurocentral). Oblique cuts in a plane at right angles to the axis of the intervertebral foramina show the dimensions of the foramina, the protrusion into foramina of osteophytes from vertebrae, Luschka or zygapophyseal joints, and the relative size and position of the nerve roots. Transverse plane serial sections show the relation of the spinal cord and nerve roots, the posterior ganglia, the dural, pial, and arachnoid investments, the diameter of the spinal canal at various levels, and the relation between the spinal canal dimension and the size of the cord. The soft tissue in the canal and the foraminal exits were examined.
(A)
and
human
seen;
ation.
The
stained.
is gross
odontoid
are
very
disc is of normal and
height
proved
destruction
throughout.
are also grossly
apparent
the
anterior
and
are inordinately
Leon Sokoloff.
posterior thin.
posteri-
cord are well
bral erosions ligaments
are
sublux-
eroded
granulomatous
Even
anterior atlas
atlanto-axial
and spinal
narrow
section
The
of the
is grossly
Meninges
The C2-3
rest
spine.
(B) arches
there
orly (arrow).
the
cervical
posterior
easily
is a histological
but all to have Verte-
(arrow).
longitudinal
Prepared
by Dr
8
BLAND AND BOUSHEY
Fig 7:
This specimen
section
of an atlas and an axis. The anterior
arch
(A)
subluxated
of the
is a histological
atlas
(left)
is in a 12
position and the posterior
has compressed
sagittal mm
arch (PI
the spinal cord at the foramen
magnum level (arrow),
ie brain stem compres-
sion. There is the remnant of disc in the lower odontoid
(arrowhead)
and both anterior
was present in all the spines studied, we believe this to be a physiologic event, peculiar to the human cervical spine and related to biomechanits. The disc demonstrates gradual disappearance of the nucleus pulposus and deposition of ligamentous, fibrocartilage-like tissue (Fig 10). In the cervical spines from people older than 60 years, the annular dissection has reached the midline, suggesting a bisected disc from uncinate process to uncinate process. The overall material in the disc has become “dry” without gel-like material, suggesting an absence of proteoglycan, and acquiring a dense, ligamentous-like character, as well as marked loss of volume of disc tissue. We propose these physiological events are a consequence of shearing planes of tissue from biophysical and biomechanical events. We frequently found pieces of hard, marginally elastic disc tissue free in the disc that conceivably could herniate.
and
posterior odontoid surfaces are eroded by rheu-
Uncinate Processes
matoid
The uncinate processes (uncus, L. a small hook) are normal bony excrescences placed laterally and posterolaterally on C3 through C7. The uncinate process preceded the evolution of the extremities and evolved from the reptilian and avian costovertebral joints, joining ribs to vertebrae. With increasing age, the uncinate processes enlarge and often flatten, losing their sharp bony characteristics (Fig 11). This osteogenic phenomenon forms a bulwark of bone laterally and posterolaterally, which, we believe, prevents disc herniation in this area. Figure 12A shows an anterior view of an articulated cervical spine from a 42-year-old man. Figures 12B, C, and D illustrate various anatomic characteristics of uncinate processes.
granuloma.
destroyed,
The
C2-3
disc is nearly
cleft all the way through,
surfaces readily apparent.
Prepared
opposing by Dr Leon
Sokoloff.
with little or no proteoglycans. Thus, after age 40 years, it is impossible to clinically herniate the nucleus pulposus as there is none. Figure 8 is a coronal section of the cervical spine of a 4-year-old child. The nucleus pulposus is clearly present in mid-disc. The uncinate processes phylogenetically represent remnants of ancient costovertebral joints in birds and reptiles. During evolution, they preceeded the development of extremities and cervical and lumbar ribless zones. By ages 9 to 14 the nucleus pulposus is far less evident and bilateral clefts have developed in the postero-lateral annulus fibrosus, medial to the growing uncinate processes of the vertebra. This cleft is the site of the alleged joints of Luschka, uncovertebral, or so-called neurocentral, joints (Fig 9). Between ages 20 and 35, the clefts gradually enlarge and dissect tissue planes toward the midline, where each finally meets its counterpart from the opposite side. Because this phenomenon
Nerve Root Exit Sites
From the level of C3-4 distal, the anterior and posterior nerve roots exit through the dural root sleeves and are below the level of the intervertebral disc by 4 mm to 8 mm. This relation is a consequence of the early formation of the nervous system, including the spinal cord and nerve roots, followed much later by rapid growth of the bony vertebral spine. With growth and lengthening of the cervical spine, physiological traction is
ANATOMY
AND PHYSIOLOGY OF THE CERVICAL SPINE
Fig 8:
(A) This specimen
is a coronal
section of a 4-year-old
nucleus pulposus in mid-disc C5-8 (arrow), uncinate
process; C =
centrum
C6-7, C7-Tl.
(vertebral
section of the cervical spine of a 4year-old three
discs shown
costovertebral
(arrow).
joints).
The
Reprinted
uncinate with
boy’s cervical
spine showing
the
0 = odontoid: N = nucleus pulposus: U =
body). (8) This specimen
is a close-up
of the coronal
boy. The nuclei pulposi are obvious in the center of the processes
permission
are well
developed
from Hall MC: Luschka’s
(homologs
of ancient
Joint. Springfield,
IL,
Charles C. Thomas, 1965.
exerted on the cord and nerve roots. The dural root sleeve exit sites are then at the level of vertebral bodies rather than at disc level. Hence, it seems unlikely that disc disease could compress cervical nerve roots because the root exit zone is below the level of the disc (Fig 13). Incidentally, virtually all dural root sleeves become fibrotic, rigid, and stiff after the age of 50 years.
Anatomy of the Anterior (Motor) Nerve Root The anterior nerve root is normally situated very low in the intervertebral foramen, preventing compression. The posterior nerve root is well protected from disc herniation. However, the zygapophyseal joints may become enlarged and osteophytic and could compress the posterior roots. We believe that radiculopathy, though
very unusual, is a consequence of zygapophyseal joints impingements, but not due to enlarged uncinate processes (so called Joints of Luschka or the discs) (Fig 14).
Relation Between Spinal Cord Volume and Bony Canal Normally there is considerable individual variation between the spinal cord volume and space available in the bony canal. This is likely a constitutional or genetic characteristic. Clinically, if one inherited a “fat” spinal cord and relatively small bony canal, the development of osteoarthritis would have a narrow margin for compromise of cord function. The ideal is thin cord and a capacious spinal canal, affording a large margin of safety (Fig 15).
BLAND AND BOUSHEY
Fig 9: (A) This specimen is of a coronal section of a 14-year-old girl’s cervical spine for comparison with Figure 8. Note postero-lateral clefts have developed in the annulus fibrosus just medial to the uncinate
process (arrows).
The uncinate
process is well developed
the uncinate process of Figure 8. The vertebral on viewer’s (arrows).
right. (8) A close-up of A illustrates
Reprinted
with
permission
from
and relatively
much larger than
artery has been cut in the coronal plane (arrowhead) the clefts in the postero-lateral
Hall MC: Luschka’s
Joint,
Springfield,
annulus fibrosus IL, Charles
C.
Thomas, 1985.
Anatomy of Anterior and Posterior Spinal Canal
In people over age 45 the anterior spinal canal is characterized by bars of osteophytes at the level of the intervertebral discs, commonly compressing the cord to varying degrees and clinically symptomatic. The ligamentum flavum is hypertrophied and hyperplastic, projecting into the spinal canal and may compress the spinal cord posteriorly. The posterior longitudinal ligament in the cervical spine is three- to fivefold thicker and more developed than in either the thoracic or the
lumbar spine, where it becomes attenuated. Because the position of the posterior longitudinal ligament prevents herniation posterolaterally and posteriorly, this may partially explain the unusual occurrence of cervical disc herniation. The anterior longitudinal ligament is attached firmly to the vertebral bodies but only loosely at the disc area. The posterior longitudinal ligament is firmly attached to the disc area but loosely to the vertebral surface. These anatomic facts may explain why osteophytes are larger and more common anteriorly than posteriorly. In the cervical region only (not in the thoracic or
11
ANATOMY AND PHYSIOLOGY OF THE CERVICAL SPINE
lumbar region), the posterior longitudinal ligament is broad throughout, while in the thoracic and lumbar region it narrows strikingly behind each vertebral body. A strange and poorly understood cervical spine characteristic is the marked remodelling, hypertrophy, and hyperplasia of cervical vertebrae that occurs with increasing chronologic age (Fig 16). Menisci of Zygapophyseal Joints
All zygapophyseal (posterior) joints have menisci in a circular arrangement with varying degrees of projection into the joint. These seem to have a propensity to proliferate in a fibrous-like pannus. Most hyaline cartilage surface is fibril-
Whole cervical spine specimen (cut Fig 11: coronally) from an 80-year-old woman. Note the striking decrease in overall disc substance, marked narrowing of all discs, completely bisected discs (arrowheads right), surfaces facing one another, in life only a potential space. Also note the large, flattened
and deformed
uncinate
posterolateral
processes
on upper,
edges of the vertebrae (arrows), a bulwark of bone that increases in volume with increasing chronologic age.
Fig 10:
A coronal section of cervical vertebrae
C4, and C5, and intervertebral adolescent dissecting
girl illustrating toward
disc from an
the annular
the midline
clefts
in collagenous
tissue planes of the anulus fibrosus (arrows). There is no evidence of synovium, subchondral bone, or joint capsule. See text.
lated with chondrocyte clone suspect the proliferation may be bilization, relative, or absolute. menisci provide the joint with 17).
formation. We related to immoWe propose the lubrication (Fig
Luschka “Joints”
Anatomically, a joint has certain structural elements, namely synovium, subsynovial space, hyaline cartilage, subchondral bone, and capsule. At the site of the alleged Luschka joints no such
Fig 12:
(A) This is an anterior
uncinate
process.
view of a normal articulated
(B) An anterior
process in the viewer’s
view of the C4 vertebrae
left and blunt remodelling
a cervical spine from an 64-year-old very large uncinate the viewer’s
processes
grown
cervical spine. The arrow illustrating
points to an
a sharp, remodelled
uncinate
uncinate process in the right. (C) A coronal view of
man. The intradiscal surfaces are shown (arrowhead). up and overlapping
the vertebrae
right is a sagittal section seen by roentgenogram.
White
There are
above (white arrow). arrows
(D) On
point to the uncinate
processes as seen from within the disc, looking from the inside out. On the left is a sagittal section of the same anatomical Note the through
specimen
and through
showing
C4 vertebrae
clefts (black arrows
permission
from Ball J: The articular pathology
Rheumatic
Diseases (ERA).
toid Arthritis.
61:25-39,
International
1964.
in the middle and the lower half of C3 above. above and below C4 vertebra).
of rheumatoid
Congress
arthritis.
International
Reprinted
with
Study Center for
Series No. 61. Radiological Aspects
of Rheuma-
ANATOMY AND PHYSIOLOGY OF THE CERVICAL SPINE
Fig 13:
(A) A sagittal
arrows than
point
to exit
section
sites
at the level of the discs.
ligaments
have
leave
spinal
having
the
been
been
laid back,
the motor
rootlets
projected
superiorly
down bulges.
(lower
severed,
canal
black
The spinal
of a whole
(dural
root
See text. allowing
at vertebral faces
(anterior) (upper arrow).
the viewer;
cord runs down
the
showing
they
the anterior the spinal while short
the middle
apart
arrows).
The
spinal
artery
exit
section, at two ventral
sites.
Note
two
of the vertebrae
the anterior levels.
Note
surface
is seen (outlined
black rather
and posterior the
nerve
roots
of the
spinal
cord,
arrow)
as well
as
cord. (Cl In this spine the upper nerve roots are
the lower open
nerve
are at the level
a sagittal
discs to spring (2 black
from
black arrow) Incidentally
spine
and that
(B) In this spine,
levels
coming
cervical
sleeves)
arrow
ones are at increasing head
points
obliquity
to posterior
from
above
disc osteophytic
of the illustration.
structures were present; therefore, it seems improper to call this region a joint. We believe the anatomic structure we describe and the physiological sequence of events we propose constitute an accurate description of cervical intervertebral disc pathophysiology. Between 9 to 14 years of age a cleft develops in the lateral and posterolateral annulus fibrosus, a function of the unusual ability to rotate the cervical spine in obligate bipeds. Rather than forming a true joint, this cleft or fissure gradually dissects toward the midline to meet the lateral cleft dissecting from the other side. In this space there are only patches of synovium. With increasing age the disc is finally bisected in a transverse plane and the two surfaces are made up of a
peculiar mix of connective tissues: anulus-like tissue, fibrocartilage, hyaline cartilage, tendonlike tissue, and bony islands. The disc becomes narrower with the passage of time, sometimes nearly disappearing. We have dubbed this the “grin of Luschka” when the process is complete (Fig 16D). This finding was universal in 17 1 the spines studied (Fig 18).
The Autonomic Nervous System A large part of our nervous system is concerned with activities and vital functions of which we are unaware and over which we have no voluntary control. Claude Bernard proposed that a divine providence considered these autonomic activities far too important to entrust to a capri-
BLAND AND BOUSHEY
Fig 14: A coronal section through the intervertebral foramen at C7-Tl. The curved arrow points to the posterior
(sensory)
straight arrow to the anterior level in the foramen root ganglion. situated tected
very from
throughout
root and the
(motor) root. The
is proximal to the posterior
The motor root is anatomically low
in the foramen,
any
osteophytic
well
pro-
compression
its course from the spinal cord. See
text. To the right of the foramen the superior, posterolateral
is the cleft in
intervertebral
disc,
the anulus fibrosus.
cious will. “We are thrust into this world by smooth muscle, which is under the control of the autonomic nervous system. From moment to moment we are dependent for our conscious existence on the moderate contraction of blood vessels, routinely kept in this state by autonomic impulses. Most of the complicated processes of digestion, from the initial outpouring of saliva to the final riddance of waste, require the participation of autonomic nerves. Any vigorous exercise in which we may engage depends on cooperation of autonomic government of appropriate effectors, thus throughout eons of past time the physical struggle for existence has been made possible by that government-which preserves the stable states of the fluid matrix that are required for ready response to every call to action.” Led by Walter B. Cannon, physiologists the world over have worked long and hard to elucidate the functions of the autonomic nervous system in maintaining “le milieu interne.” Only
in the last two decades has research attention been paid to the manner in which the autonomic nervous system participates in neurologic diseases, or how the sympathetic-parasympathetic nervous system may be selectively deranged by pathological processes. Pertinent to the cervical spine and its clinical syndromes is the fact that no preganglionic sympathetic fibers are present in the neck. All neck fibers come from Tl, 2, and 3 levels, having their first synapse in one of the three cervical sympathetic ganglia, stellate, middle, and superior. The postganglionic fibers go in three directions. (1) Into the upper extremities providing all autonomic functions, circulatory vasomotion, sweating, proprioception. (2) Reenter the spinal cord through the intervertebral foramina and have synaptic connections in the vestibular apparatus, cerebellum, thalamus and hypothalamus. (3) Follow the distribution of the vertebral and carotid arteries in the brain. The earliest description of sympathetic nervous system syndromes was by Barre’* in 1926, and further described by his student Lieott” in 1928. So diverse and bizarre were the symptoms and signs that some authors did not associate them with the cervical spine.*‘**’ The current view is that the nebulous and bizarre complaints are explicable on the basis of known autonomic, neurologic, and pathophysiologic events. The clinical results relate to the interplay between mechanical derangements of the cervical spine and the cervical sympathetic system, the brain and spinal cord, the vertebral artery, the zygapophyseal joints, the cervical vertebrae, the scalene muscle system and preexisting arteriosclerosis.22T23 Though it was known by the ancient Egyptians of the 16th and possibly the 30th century that spinal cord injury involved more than just motor and sensory loss, a full grasp of the physical and physiological complexities of autonomic functional alteration is lacking. Nevertheless, we do have valid clinical, physiological explanations for such common cervical spine occurrences as cervical dizziness and ataxia; nystagmus; various syndromes of loss of proprioception, cervicoocular, and vestibulo-ocular reflexes; pupillary dilation on neck extension; postural and pathological gaits induced by cervical spine disease; and
ANATOMY
Fig 15: (white
(A) arrow)
A transverse and the
without
cord
showing
a grossly
arrow
heads),
section
large
The three
and spinal
cervical
canal.
(D)
C6 vertebra
spinal
cord,
myelopathy. cord
From
cervical
spines
middle
sections
(arrows)
left
of osteoarthritis.
relatively
illustrating
from
to right
a series
thin
very
through
constricting
spinal
sizes of spinal
spinal
change.
cervical
spine
specimens.
and
and spinal
spine (white sagittal
a relatively
These
24 on left to 89 years cords
canal
(C) A gross
(arrow)
canal
osteoarthritis
a whole
cervical
cord
of six different in age from
spacious
for extensive
mild osteoarthritic
ranging varying
the
tolerance
sections
narrow,
a rather
patients
illustrate
illustrating
ie, a great
(B) Coronal
and a relatively
for even spine
of whole
magnitudes
through
small
ie, an intolerance
spinal
sections
section
relatively
compression
of a whole
capacious
varying
15
AND PHYSIOLOGY OF THE CERVICAL SPINE
sagittal
on the right.
canals
as well
as
See text.
ataxia during the Romberg test.24*25Table 1 lists autonomic symptoms and signs arising from pathophysiologic changes in the cervical spine. SUMMARY
The following findings derive from studying 171 whole human cervical spines. (1) The nucleus pulposus, present at birth, is absent in the adult cervical spine. (2) The uncinate process,
present at and before birth, gradually enlarges superiorly, forming a lateral and posterolateral bulwark of bone, preventing herniation of disc material. (3) The posterior longitudinal ligament is four- to fivefold thicker in the cervical spine than in the thoracic or lumbar spines, probably preventing posterior herniation. (4) From C3-4 the nerve root exit sites are below the disc level, explained by bone growth exceeding spinal cord
Fig 16: (A) On the left, the specimen illustrates the posterior surface of cervical vertebra seen from inside the spinal canal, showing massive posterior osteophytes of the intervertebral discs, protruding into the anterior cervical spinal canal (arrows). The posterior longitudinal ligament is greatly thickened. On the right is a section illustrating the posterior surface of the spinal canal with immensely thickened and protruding ligamenta flava (arrows). (B) A whole cervical spine cut sagittally down the midline, dividing spine and spinal cord into two facing halves. The white spinal cord is in mid section. The upper left black arrow points to a greatly hypertrophied and hyperplastic anterior longitudinal ligament and very narrow disc. The lower arrow points to an extremely thickened posterior longitudinal ligament and large osteophyte compressing the spinal cord anteriorly. On the right, the white arrow points to severely thickened ligamenta flava compressing the spinal cord posteriorly. (C) A cervical spine cut from the lateral aspect exposing the spinal cord with its nerve rootlets and anterior and posterior nerve roots down the middle; P, posterior; A, anterior. To the left of the spinal cord is the dura mater and the hypertrophied ligamenta flava (arrow). To the right of the spinal cord is the anterior surface of the spinal canal showing the hypertrophied anterior longitudinal ligament and large posterior osteophytes at disc levels compressing the anterior spinal cord (arrows). The spinal cord has multiple compression sites. (D) A coronal section of a cervical spine from an 68-year-old man. Note the increasing bulk of the remodelled vertebrae from above downward; each larger than its neighbor above, representing extreme, presumably physiologic remodelling, hypertrophy and hyperplasia with increasing chronologic age. Incidentally note large, deformed and flattened uncinate processes (black arrow). cleavage of all the discs with very little disc elements remaining. desiccated, pebbly to knobbly disc surfaces facing one another (white arrow).
ANATOMY
Fig 17:
AND
PHYSIOLOGY
OF THE CERVICAL
SPINE
(A) Sagittal section of tygapophyseal
(see text) and the upper arrow proliferating
joints. The lower arrow points to a normal meniscus
points to a proliferating
meniscus.
meniscus of Figure 17A. upper arrow. It is a fibrous-like
hyaline cartilage (C) surface. (Cl An illustration
of a zygapophyseal
(B) A histological
section of the
pannus (PI proliferating
over the
joint held open by the examiner.
The hyaline cartilage surfaces are seen. In the joint, the arrow points to a normal meniscus. We found circular menisci in virtually all cervical zygapophyseal joint. The joint space (JSI is in the upper thickened
subchondral
joints. (D) A section through
third of the illustration.
bone (B). the hyaline cartilage having been completely
the bone and tightly adherent
to it is the peculiar proliferating
we believe, from the meniscus.
a zygapophyseal
The lower third shows
fibrous-like
destroyed.
dense,
Overlying
pannus (brackets) deriving,
Fig 18: (A) A histological coronal section of the superior posterolateral anulus fibrosis of a cervical disc from a 14year-old girl. The cleft is dissecting in tissue planes, medially to meet its counterpart from the opposite side. The upper arrow points to the cleft. There are patches of synovial-like tissue above the arrow but no evidence of other components of a true joint. The lower arrow points to the dissecting cleft proceeding medially. See text. (8) This illustration is a coronal section of the uncinate process KJ)from a 28-year-old man. The cleft or fissure (F) medial to the uncinate process shows disc fibrocartilage fibrillation (arrow) but no evidence of synovium, capsule, subchondral bone or hyaline cartilage, normal components of a true joint. We suggest that the hyaline cartilage which Luschka, and many others since, described was that of the cartilage end plate of the intervertebral disc, a normal finding noted in this illustration. (C) This illustration (54-year-old man) of a sagittal section midline of the cervical spine shows uniformly very narrow intervertebral discs, loss of most of the disc tissue and the clefting or fissures have dissected all the way across the disc (arrow). (D) This illustration (72-year-old woman) of a sagittal section of a cervical spine shows the anterior and posterior ligaments cut allowing the anatomically bisected disc to spring apart disclosing the knobbly, irregular internal surfaces with some loose fibrocartilage pieces. The arrow points to one. Presumably these could herniate. (E) This illustration of a sagittal section of a cervical spine is cut so that the internal surfaces of the completely transected intervertebral discs may be viewed (arrows). Histologically these surfaces are a mixture of hyaline cartilage, fibrocartilage, bony fragments and fibrotic nodules, usually with some loose pieces. These findings were nearly universal in varying degree in spines from patients over age 45 to 50 years.
19
ANATOMY AND PHYSIOLOGYOF THE CERVICAL SPINE
Table 1. Symptoms and Indirectly
From
and Signs Arising Directly Tissues
in the
Cervical
Spine Signs
Symptoms Pain
Falling
Headache
Tender scalp
Dizziness
Tender bones
Vertigo
Anesthesia
Paresthesia
Hyperesthesia
Fatigue
Dysesthesia
Insomnia
Atrophy
Restless arms
Hypertrophy
and legs
Hyperplasia
Cough
Weakness upper extremity
Sneeze
Asymmetry
Nausea and vomiting
Sweating (or lack of)
Diarrhea
Nystagmus
Fainting
Tender muscle
Visual disturbances
Fasciculation
Auditory disturbance
Transient hearing loss
Drop attack
Spastic gait
Arm and leg ache
Reflex changes (deep ten-
and pain Stiff neck
don, superficial and auCarotid sinus sensitivity
Pathological gait
Proprioceptive loss
Poor balance
Benign paroxysmal posi-
Muscle twitch Mood depression Tinnitus Diplopia
ACKNOWLEDGEMENTS
tonomic reflexes)
Torticollis
Speech disturbance
growth resulting in “traction” on the spinal cord and nerves and increasing downward obliquity of the roots. Discs, normal or pathological, cannot compress nerve roots. (5) Joints of Luschka are nonexistent. (6) A cleft appears in the posterolatera1 anulus fibrosus at the age of 9 to 1.5 years and dissects medially from both sides in adolescence and young adult life, bisecting the disc with a potential space, sparsely lined with synovium. (7) The anterior nerve roots are anatomically too low in the intervertebral foramen to be subject to compression. Except for zygapophyseal joint osteoarthritis, the posterior nerve roots are remarkably well protected. Radiculopathy is an uncommon clinical phenomenon, (8) There are no preganglionic autonomic nerve fibers in the cervical spine, all arising from Tl, 2, and 3 levels, first synapsing in the stellate, mid, and superior cervical autonomic ganglia. (9) Myelopathy is a far more common clinical event than radiculopathy.
tional nystagmus (BPPN) Vertebro-basilar insufficiency Sensory ataxia Physiological head extension vertigo Cervico-collie reflex Cervico-ocular reflex Vestibulo-ocular reflex
The authors wish to make the following acknowledgements with gratitude. To Mary S. Skovira for her superb, painstaking; and ever-prompt completion of all manuscript, bibliographies, and illustrative material over the past 15 years. To Professor Dallas R. Boushey, anatomist, for excellent disseetions and education of the authors as to what was known anatomy and what anatomy had not been described. To Dr Leon Sokoloff for teaching the authors how to prepare cervical spines for gross and microscopic study and the preparation of faultless pathological specimens. To Professor John Ball for educating the author in rheumatoid arthritis and osteoarthritis pathology in many long, exciting, and always amusing discussions; he also taught the method of removing cervical spine in order to derive the best and most from anatomical and pathological studies.
REFERENCES
1. Hadler NM: Osteoarthritis as a public health problem. Clin Rheum Dis 11:175-185, 1985 2. Lawrence JS: Disc degeneration; its frequency and relationship to symptoms. 28:121-137, 1969 3. Hult L: The Munkfors investigation. Acta Orthop Stand 16:1-76, 1954 (suppl) 4. Hult L: The cervical, dorsal and lumbar spine syndromes. Acta Orthop Stand 17:1-102, 1956 (suppl) 5. British Association of Physical Medicine: Pain in the arm and neck: A multicentre trial of the effects of physical therapy. Br J Med l:253-258, 1966 6. Brain WR: Discussion on rupture of the intervertebral disc in thecervical region. Proc R Sot Med 61:509-516, 1948 7. Hadler NM: Regional musculoskeletal diseases of the
low back-Cumulative trauma versus single incident. Clin Orthop201:1019-1025, 1987 8. Hadler NM: Medical management of the regional musculoskeletal diseases. Philadelphia, PA, Grune and Stratton, 1984 9. Frymoyer JW, Donaghy RMP: The ruptured intervertebral disc: A 50 year follow up of the first case. J Bone Joint Surg67A:1113-1116,1985 10. Mixter WJ, Barr JS: Rupture of the intervertebral disc with involvement of the spinal canal. N Engl J Med 211:210-215, 1934 11. Hadler NM: Illness in the work place; the challenge of musculoskeletal symptoms. J Hand Surg lOA:451-456, 1985 12. Hadler NM, Gilling DB, (eds): Arthritis and Society:
20
The Impact of Musculoskeletal Disease (vol 3). London, England, Butterworths, 1985 13. Tondury G: The cervical spine, its development and changes during life. Act Orthop Belg 25602-626, 1959 14. Payne EE, Spillane JD: The cervical spine. An anatomical, pathologic study of 70 specimens with particular reference to cervical spondylosis. Brain 80:571-596,1957 15. Holt S, Yates PO: Cervical spondylosis and nerve root lesions. J Bone Joint Surg 48B:407-423, 1966 16. Frykholm R: Cervical nerve root compression resulting from disc degeneration and rcot sleeve fibrosis. A clinical investigation. Acta Chirurgia Stand Suppl 160:1-49, 1950 17. Hirsch C, Schajowicz F, Galante J: Structural Changes in the Cervical Spine. Copenhagen, Denmark, Munksgaard, 1967 18. Barre J: Le syndrome sympathique cervical posterieur et sa cause for frequent l’arthrite cervicale. Rev Neurol 33:1246-1254, 1926 19. Lieou YC: Syndrome sympathique cervical posterieur
BLAND AND BOUSHEY
et arthrite cervicale chronique, in Etude Clinique et Radiologique. Strasbourg, France, Schuler and Minh, 1928 20. Kovaks A: Subluxation and deformation of the apophyseal joint: A contribution to etiology of headache. Acta Radiol43:1-6, 1955 21. Rotes-Queral J, Crespi PB, Pariggros AC: Studies on locomotor syndromes of possible psychogenic origin II. The so-called Barre-Lieou syndrome. Med Clin (Bare) 33:235246,1949 22. Bland JH, Boushey DR: Disorders of the Cervical Spine. Philadelphia, PA, Saunders 1987, pp 182-224 23. Stewart DY: Current concepts of the Barre syndrome or the posterior cervical sympathetic syndrome. Clin Orthop 24:40-48, 1980 24. Hines S, Houston M, Robertson D: The clinical spectrum of autonomic dysfunction. Am J Med 70:20911096,198l 25. deJong JMBV, Bles W: Cervical dizzyness and ataxia. In Bles W (ed): Disorders of Posture and Gait. Elsevier Science, Amsterdam, The Netherlands, 1986