Syringomyelia, arachnoiditis

and spasticity
Dr. Samuel Oluka

Supervisor: Dr. Kiboi

Syringomyelia
Defn:
• Cystic dilation within the substance spinal cord

Hydromyelia:
• is a dilatation of the central canal by cerebrospinal fluid (CSF

• Olivier d’Angers in 1827

• Greek syrinx, meaning a “cavity of tubular shape,” and myelos, meaning “marrow
Syringomyelia
cystic cavitation of the spinal cord that contains fluid similar to cerebrospinal fluid
(CSF) and is lined by gliotic tissue, or, in some circumstances, ependymal cells

• NB: the term has been used to describe several types of intramedullary cysts,

Proteinaceous cysts,
Terminal ventricles
Hydromyelia
Terminal ventricle
• ependymal-lined fusiform
dilatation of the terminal central
canal of the spinal cord,
positioned at the transition from
the tip of the conus medullaris
to the origin of the filum
terminale
INCIDENCE AND EPIDEMIOLOGY
• Largely affects children and young adults.
• The prevalence has been estimated at 9 per 100,000 people,
• Incidence of 0.44 cases per year has been cited in the literature

• These figures likely underestimate the true prevalence and incidence because these estimates were derived from data
collected in the pre–magnetic resonance imaging (MRI) time period

• 130 per 100,000 has been reported in certain regions of Russia,

• Possibility of regional and ethnic variation in the prevalence of this condition
INCIDENCE AND EPIDEMIOLOGY
• Chiari malformations are responsible for nearly half of the
affected population,

• While spinal cord trauma and arachnoiditis account for another quarter of adult patients
with syringes.

• Up to 64% of patients who suffer spinal cord trauma will develop a syrinx;

• however, only 1% to 9% of spinal cord injured patients will become symptomatic from the
syrinx

• Other causes of syringes include intramedullary tumors, degenerative disease, arachnoid

webs, and spinal cord tethering
PATHOPHYSIOLOGY
1. The hydrodynamic theory

2. William's theory

3. Oldfield's theory

4. The intramedullary pulse pressure theory

1. Gardner's hydrodynamic theory
• Proposes that syringomyelia results from a "water hammer"-like
transmission of pulsatile CSF pressure via a communication between
the fourth ventricle and the central canal of the spinal cord through
the obex.

• A blockage of the foramen of Magendie initiates this process.

• initially postulated by Gardner in the 1960s

2. William's theory
• Syrinx development, particularly in patients with Chiari malformation, follows
a differential between intracranial pressure and spinal pressure caused by a
valve-like action at the foramen magnum. 
• The increase in subarachnoid fluid pressure from increased venous pressure
during coughing or Valsalva maneuvers is localized to the intracranial
compartment.
• The hindbrain malformation prevents the increased CSF pressure from
dissipating caudally.
• During Valsalva, a progressive increase in cisterna magna pressure occurs
simultaneously with a decrease in spinal subarachnoid pressure.
• This craniospinal pressure gradient draws CSF caudally into the syrinx.
3. Oldfield's theory
• Downward movement of the cerebellar tonsils during systole can be
visualized with dynamic MRI.
• This oscillation creates a piston effect in the spinal subarachnoid space that
acts on the surface of the spinal cord and forces CSF through the
perivascular and interstitial spaces into the syrinx raising intramedullary
pressure.
• Signs and symptoms of neurological dysfunction that appear with distension
of the syrinx are due to compression of long tracts, neurons, and
microcirculation.
• Symptoms referable to raised intramedullary pressure are potentially
reversible by syrinx decompression. 
4. The intramedullary pulse pressure
theory
• syringomyelia is caused by increased pulse pressure in the spinal cord
and that the syrinx consists of extracellular fluid.

• A new principle is introduced implying that the distending force in the

production of syringomyelia is a relative increase in pulse pressure in
the spinal cord compared to that in the nearby subarachnoid space.

• The formation of a syrinx then occurs by the accumulation of

extracellular fluid in the distended cord.
 Class Type of syngomyelia
I Communicating syringomyelia
Central canal dilations
Classification 1. Communicating hydrocephalus (posthemorrhagic,
postmeningitic)
2. Complex hindbrain malformations (Chiari type II,
encephalocele)
• Milhorat 3. Dandy-Walker malformation
developed a II Noncommunicating syringomyelia
classification of Central canal/paracentral syringes
1. Chiari malformation
syringomyelia 2. Basilar invagination
3. Spinal arachnoiditis (posttraumatic, postmeningitic)
based on 4. Extramedullary compression (spondylosis, tumors, cysts)
pathophysiology 5. Tethered cord
6. Acquired tonsillar herniation (hydrocephalus, intracranial mass
lesions)
Primary parenchymal cavitations
1. Spinal cord trauma
2. Ischemia/infarction
3. Intramedullary hemorrhage

III Atrophic cavitations (syringomyelia ex vacuo)

IV Neoplastic cavitations
CLINICAL PRESENTATION
Initial symptoms:
• Sensory loss and dull or burning pain attributable to spinothalamic tract
involvement.

• Dissociated pattern of pain and temperature sensation loss with preservation of

light touch and proprioception

• Loss of motor function occurs following sensory loss with progression of the
syrinx; weakness tends to be asymmetrical because of asymmetrical extension of
the syrinx into the ventral horn of the spinal cord
further progression
• Hyperhidrosis
• Autonomic dysreflexia
• Horner’s syndrome
• Asymmetrical reduction of reflexes

• Progression is generally slow, although sudden worsening has been reported in

posttraumatic syringomyelia as a result of hemorrhage into the syrinx cavity

• clinical stabilization has occasionally been reported after progression by some

authors
Syringobulbia
• When the syrinx is extensive or in the high cervical region, it may sometimes extend into the
caudal brainstem and result in lower cranial neuropathies;

• The clinical presentation is highly dependent on the location

of the syrinx.

• Patients with arachnoiditis more commonly present

with signs and symptoms attributable to lower lumbar and sacral
spinal segments, because arachnoiditis is more prevalent in the
lumbar spine owing to its association with myelography.
• In contrast, patients with posttraumatic syrinx and complete spinal
cord injuries may develop a gradual loss of function at progressively higher spinal levels
RADIOLOGIC EVALUATION
• If syringomyelia is noted, MRI of the entire neuraxis is indicated in order to rule out other
associated conditions, such as foramen magnum pathology or spinal cord tethering.
• Posttraumatic syringes should not enhance following the administration of gadolinium, and the
signal characteristics of the syrinx fluid should be similar to those exhibited by CSF
• Phase-contrast cine MRI may aid in localizing subarachnoid space obstruction, it may
demonstrate normalization of CSF flow if surgery is performed.

• It is also useful to note that computed tomography (CT) is not a reliable modality to evaluate
for syringomyelia;

• CT myelography does not detect up to 50% of syringes

ETIOLOGY
A. Bony abnormalities include the following:
• Small posterior fossa
• Platybasia and basilar invagination
• Assimilation of the atlas
B. Soft-tissue masses of abnormal nature include the following:
• Tumors (eg, meningioma at foramen magnum)
• Inflammatory masses
C. Neural tissue abnormalities include the following:
• Cerebellar tonsils and vermis herniation
• Chiari malformation
D. Membranous abnormalities include the following:
• Arachnoid cysts , rhombic roof, or vascularized membranes
• Posthemorrhagic or postinflammatory membranes
E. Other etiologies not associated with craniovertebral abnormalities
• Arachnoid scarring related to spinal trauma
• Arachnoid scarring related to meningeal inflammation
• Arachnoid scarring related to surgical trauma
• Subarachnoid space stenosis due to spinal neoplasm or vascular malformation
• Subarachnoid space stenosis, with possible scarring, related to disk and
osteophytic disease
• Idiopathic
Posttraumatic Syringomyelia
• Nearly 50% develop spinal cystic change and between 21% and 28%
develop a syrinx over three decades of follow-up after injury.
• However, only 1% to 9% of patients with SCI will progress to
symptomatic syringomyelia.
Pathogenesis of Post traumatic
Syringomyelia
1. inflammatory responses to the initial traumatic injury result in
localized spinal cord edema and cyst formation
2. Intramedullary hematoma, when present, may leave a cystic cavity
after absorption of the hematoma and may increase
the likelihood of syrinx formation
3. Predilection for vascular watershed regions of the spinal cord,
suggesting that ischemia resulting from the primary injury or as a
secondary injury caused by the subsequent inflammatory response may
play a role in the initiation of posttraumatic syringomyelia.
4. Subarachnoid adhesions, deformity, and spinal stenosis are
commonly seen in association with SCI and may contribute to the initial
formation and progression of syringomyelia via interruption of normal
CSF flow in the subarachnoid space
Treatment
Difficult to manage
1. Remain asymptomatic for several years even with significant
syringes.
2. Surgical treatment is generally deferred until the patient
develops a progressive neurological decline or severe pain.
Suboccipital And Cervical Decompression
• This operation includes suboccipital craniectomy; laminectomy of C1,
C2, and sometimes C3; and duraplasty.

• Some authors report microsurgical lysis of any adhesions, opening of

the fourth ventricular outlet, and plugging of the obex

• (later steps are based on Gardner's hydrodynamic theory).

Laminectomy And Syringotomy (Dorsolateral Myelotomy)

• After decompression, the syrinx is drained into the subarachnoid

space through a longitudinal incision in the dorsal root entry zone
• (between the lateral and posterior columns), usually at the level of C2-C3.

• Incision in the dorsal root entry area has the minimum risk of
increasing neurological deficit.
Shunts
1. Ventriculoperitoneal shunt - Indicated if ventriculomegaly and
increased intracranial pressure are present
2. Lumboperitoneal shunt - Placed infrequently because of increased
risk of herniation through the foramen magnum
3. Syringosubarachnoid dorsal root entry zone shunt
4. Syringoperitoneal shunt
Percutaneous needling
• This technique is advocated as a possible mode of therapy; however,
rapid refilling of the hydromyelic cavity from the ventricular system
follows aspiration of fluid at the time of surgery.

• Moreover, a needle track seems unlikely to remain open.

Terminal ventriculostomy
• The terminal ventricle is the dilated portion of the central canal that
extends below the tip of the conus medullaris into the filum
terminale.
• A laminectomy is performed over the caudal limit of the fluid sac, and
the filum is opened.
• This procedure is suitable only in patients with symptoms of syrinx
without Chiari malformation.
• It is inappropriate in cases in which the hydromyelic cavity does not
extend into the lumbar portion of the spinal cord or into the filum
terminale.
Neuroendoscopic surgery
• This technique is particularly useful in evaluating and treating multiple
septate syrinxes.
• A fibroscope inserted through a small myelotomy allows inspection of
the intramedullary cavity.
• Septa are fenestrated, either mechanically or by laser.
• Fluid from the cavity is then shunted into the subarachnoid space.

Surgical untethering in select cases with posttraumatic tethering

associated with syringomyelia
ARACHNOIDITIS
Defn:
•  denotes inflammation of the meninges and subarachnoid space.

• It is characterized by thickening of the arachnoid membrane and dura

mater adhesions that result in chronic lower back pain
Causes
Infectious
Inflammatory
Neoplastic processes.

Infectious causes include bacterial, viral, fungal, and parasitic agents.

(bacteria, tuberculosis, cysticercosis, candidiasis, chromoblastomycosis, cryptococcosis, listeriosis, syphilis)
Noninfectious inflammatory
• Etiologies include

Surgery

Intrathecal haemorrhage

Administration of intrathecal agents, such as myelographic contrast

media, anesthetics, and steroids
Neoplasia
• Includes the hematogenous spread of systemic tumors, such as breast
and lung carcinoma, melanoma, and non-Hodgkin lymphoma.

• Neoplasia also includes direct seeding of the cerebrospinal fluid (CSF)

from primary central nervous system (CNS) tumors
Medulloblastoma
Ependymoma
Choroid plexus carcinoma.
Spinal Adhesive Arachnoiditis (SAA)
• Refers to scarring of pial and arachnoid membranes within the thecal sac and
occurs as a sequela of chronic inflammation
• The severity of disease ranges from focal adhesions to dense scarring
• Causative triggers of SAA include infection, history of myelography, subarachnoid
hemorrhage, degenerative lumbar disease, previous spine surgery, and intrathecal
steroid injection
• Onset of symptoms can be variable and ranges from only 14 days to up to 44 years
following the initial insult, delayed onset is typical, with most symptoms
developing years after the inciting event
• many patients with SAA remain asymptomatic despite radiographic
findings of SAA.

• Some authors have reported radiographic evidence of SAA in 16.5% to

35% of patients who underwent myelography.

• In contrast, the incidence of symptomatic lumbar arachnoiditis after

myelography has been estimated at approximately 1%
• SAA has a predilection for the lumbar spine

• Because of this predilection, patients often initially present with

lumbar radiculopathy and back pain in addition to the signs and
symptoms attributable to a syrinx noted beforehand
Classification
• Ross and Delamarter classification based on MRI findings

1 Conglomeration of adherent nerve roots residing

centrally within the thecal sac
2 Peripherally adherent nerve roots and “empty thecal
sac” appearance, meningeal thickening
3 Soft tissue mass within spinal canal with obliteration
of the subarachnoid space
Arachnoiditis ossificans
• is considered a sequela of chronic arachnoiditis where there is a
development of calcification-ossification of the arachnoid membrane
usually of the thoracic and lumbar spines

• It is an extremely rare cause of spinal canal stenosis and consequent

neurological compromise.
Pathology
• Its exact aetiology is uncertain although thought to be likely due
osseous metaplasia associated with chronic inflammation.

• It is often associated with prior surgery or dural violation.

Radiographic features
• It may be seen as areas of linear or mass-like intrathecal lesions

CT
• Can be seen as calcification-ossification within the spinal canal
MRI
• T1: intrathecal ossification is hyperintense
• T2: intrathecal ossification is hypointense
Treatment and prognosis
• If progressive neurologic deficits occur, decompression via
laminectomy may prove successful
Spasticity
 Characterized by
Defn: increased muscle tone,
velocity-dependent increase increased intermittent or
in muscle resistance
sustained involuntary somatic
 Result of insult to the
reflexes
central nervous system or clonus
motor neurons
muscle spasms (in some patients, painful)
in response to stretch and/or
noxious cutaneous stimulation
Etiology
• primary cuause • secondary causes
• Degenerative conditions
such as spinal cord injury

Trauma to the brain

inflammatory conditions such

as multiple sclerosis.
Signs and symptoms
• resistance to a passive change in Distal flexor muscles of the
a joint angle. lower extremities

• most commonly noted in:

Flexor muscles of the upper
extremities
Proximal extensor muscles of
the lower extremities
Patterns

Cerebral palsy Cerebral palsy, stroke, or TBI

• Diplegic pattern: Adduction and internal rotation of the
• Scissoring, crouching, and toe walking shoulder

• Quadriplegic pattern:
Diplegic patterning in addition to flexion of the elbow, flexion of
Flexion of the elbow and wrist
the wrist and fingers, adduction of the thumb, and internal
rotation, pronation, or adduction of the arms
Pronation of the forearm
• Hemiplegic pattern:
Plantar flexion of the ankle, flexion of the knee, adduction of the
hip, flexion of the wrist and finger, adduction of the thumb, and Flexion of the fingers and adduction of
flexion, internal rotation, pronation, or adduction of the arms the thumb
Mechanisms
• The contraction of agonist muscles must
• The stretch reflex arc is the basic neural circuit
simultaneously be complemented by the
that underlies the problem of spasticity.
relaxation of antagonist muscles,
which occurs via an inhibitory neuron within
• The arc consists of an afferent and an the spinal cord gray
efferent limb. matter.
• This basic loop is modulated by multiple
• The afferent limb originates in the muscle synaptic influences that include descending
cerebral pathways and various interspinal
spindle and is carried in sensory neurons to the
neurons.
dorsal horn of the spinal cord.
• In the animal model, it has been demonstrated
that spasticity results, in part, in response to
• Here, synapse with a motor neuron occurs and increased glutamatergic signaling to the
the efferent limb exits via the anterior spinal uninhibited motoneurons below the level of
root innervating the contractile muscle fibers. the injury.
• The treatment of spasticity aims to interrupt
the arc at one or more points.
Assessment Ashworth
Score
Degree of Muscle Tone

• Several measures of 1 No increase in tone

hypertonicity exist
2 Slight tone increase causing a catch during
Ashworth Scale flexion or extension

3 More marked increase in tone, but readily

flexed

4 Considerable increase in tone with difficult

passive movement

5 Affected region rigid in flexion or

extension
Treatment
• multidisciplinary activity
• management for each patient is
individualized
Physical Modalities
• Passive range-of-motion • A stretching program can be
exercises reduce complemented by adjunctive
motion-triggered spasticity techniques such as tendon
vibration, muscle cooling,
postural adjustments, limb
• Static stretching using splints or loading, pressure exerted over a
serial casting can reduce stretch tight muscle belly, and electrical
reflex activity and contractures. stimulation of nerves and
muscles.
Medications • second tier of medications (demonstrated
varying levels of effectiveness)
• The most commonly used medications, including:
with
clinical trials demonstrating their Dantrolene sodium (decreases the amount of Ca2+ released
efficacy: from the sarcoplasmicreticulum)

Baclofen (GABA analogue) Gabapentin (modulating Ca2+ channels )

Tizanidine (benzodiazepine) Pregabalin

Clonidine (α2-agonist)
Botulinum neurotoxin (Chemical Blocks)
Surgical Intervention
Reserved for the most refractory • More destructive procedures
cases, such as
• myelotomy,
a. tendon lengthening, • cordotomy,
b. tenotomy, • cordectomy
c. tendon transfer.
interrupt the reflex arc, but results
are variable