Background:
Syringomyelia is the development of a fluid-filled cavity or syrinx
within the spinal cord. Hydromyelia is a dilatation of the central canal by
cerebrospinal fluid (CSF) and may be included within the definition of
syringomyelia.
Types of syringomyelia:
Syringomyelia with fourth ventricle communication
About 10% syringomyelia cases are of this type. MRI can resolve this
communication. In some cases, blockage of CSF circulation occurs. A shunt
operation may be the best therapeutic option for these patients.
Syringomyelia due to blockage of CSF circulation (without fourth
ventricular communication)
Representing at least 50% of all cases, this is the most common type of
syringomyelia. Obstruction of CSF circulation from the basal posterior fossa to
the caudal space may cause syringomyelia of this type. The most common example
is Arnold-Chiari malformation, which is also associated with communicating
syringomyelia. Other etiological causes include the following:
1) Basal arachnoiditis (postinfectious, inflammatory, postirradiation, blood
in the subarachnoid space)
2) Basilar impression or invagination
3) Meningeal carcinomatosis
4) Pathological masses (arachnoid cysts, rheumatoid arthritis pannus,
occipital encephalocele, tumors).
Syringomyelia due to spinal cord injury
Less than 10% of syringomyelia cases are of this type. Mechanisms of injury
include (1) spinal trauma, (2) radiation necrosis, (3) hemorrhage from aneurysm
rupture, arteriovenous malformation or in a tumor bed, (4) infection (spinal
abscess, human immunodeficiency virus, transverse myelitis), and (5) cavitation
following ischemic injury or degenerative disease.
Syringomyelia and spinal dysraphism
Spinal dysraphism may cause syringomyelia through a variety of mechanisms,
including those mentioned under the previous 3 categories. Identification and
treatment of associated dysraphism has the greatest impact on arresting
progression of syringomyelia.
Syringomyelia due to intramedullary tumors
Fluid accumulation is usually caused by secretion from neoplastic cells or
hemorrhagic. The most common tumors associated with syringomyelia are ependymoma
and hemangioblastoma. Extramedullary intradural and extradural tumors are
considered separately under the second category because the mechanism of syrinx
formation is by blockage of the CSF pathway.
Idiopathic syringomyelia
Idiopathic syringomyelia has an unknown cause and cannot be classified under
any of the previous categories.
Pathophysiology: Although many mechanisms for syrinx
formation have been postulated, the exact pathogenesis is still unknown.
Frequently sited theories are those of Gardner, William, and Oldfield.
Gardner's hydrodynamic theory
This 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.
William’s theory
This theory proposes that syrinx development, particularly in patients with
Chiari malformation, follows a differential between intracranial pressure and
spinal pressure caused by a valvelike action at the foramen magnum. The increase
in subarachnoid fluid pressure from increased venous pressure during coughing or
Valsalva maneuvers are 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.
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 neurologic dysfunction that
appear with distension of the syrinx are due to compression of long tracts,
neurons, and the microcirculation. Symptoms referable to raised intramedullary
pressure are potentially reversible by syrinx decompression.
Frequency:
- In the US: Estimated prevalence of the disease is about
8.4 cases per 100,000 people.
- Internationally: No difference in the prevalence of
syringomyelia among people within different geographical distributions exists.
Mortality/Morbidity:
- One half of the patients were clinically stable for several years.
- About 20% of all patients died at an average age of 47 years.
Breed: n/a
Race:
- Occurrence of syringomyelia in different races is unknown.
- Familial cases have been described.
Sex:
- Syringomyelia occurs more frequently in men than in women.
Age:
- The disease usually appears in the third or fourth decade of life, with a
mean age of onset of 30 years.
- Rarely, syringomyelia may develop in childhood or late
adulthood.
History:
Syringomyelia usually progresses slowly; the course may extend over
many years. A more acute course may occur, especially when the brain stem is
affected (ie, syringobulbia). Syringomyelia usually involves the cervical area.
Symptomatic presentation depends primarily on the location of the lesion within
the neuraxis. Clinical manifestations include the following:
- Dissociated sensory loss- Syrinx interrupts the decussating
spinothalamic fibers that mediate pain and temperature sensibility,
resulting in loss of these sensations while light touch, vibration, and
position are preserved.
- When the cavity enlarges to involve the posterior columns, there is loss
of position and vibration sense in the feet; astereognosis may be noted in
the hands.
- Pain and temperature sensation may be impaired in either or both arms,
or in a shawl-like distribution across the shoulders and upper torso
anteriorly and posteriorly.
- Dysesthetic pain, a common complaint in syringomyelia, usually involves
the neck and shoulders, but may follow a radicular distribution in the arms
or trunk. The discomfort, which is sometimes experienced early in the course
of the disease, generally is deep and aching and can be severe.
- Syrinx extension into the anterior horns of the spinal cord will damage
motor neurons and cause diffuse muscle atrophy that begins in the hands and
progresses proximally to include the forearms and shoulder girdles.
Claw-hand may develop.
- Respiratory insufficiency, which is usually related to changes in
position, may occur.
- Impaired bowel and bladder functions usually occur as a late
manifestation.
- Sexual dysfunction may also develop.
- Horner syndrome may appear, reflecting damage to the sympathetic neurons
in the intermediolateral cell column.
- A syrinx may extend into the medulla, producing a syringobulbia. This
syndrome is characterized by dysphagia, nystagmus, pharyngeal and palatal
weakness, asymmetric weakness and atrophy of the tongue, and sensory loss
involving primarily pain and temperature sense in the distribution of the
trigeminal nerve.
- Rarely, the syrinx cavity can extend beyond the medulla in the brain
stem into the centrum semiovale (syringocephalus).
- Lumbar syringomyelia can occur and is characterized by atrophy of the
proximal and distal leg muscles with dissociated sensory loss in the lumbar
and sacral dermatomes. Lower limb reflexes are reduced or absent. Impairment
of sphincter function is common.
- Painless ulcers of the hands are frequent. Edema and hyperhydrosis can
occur due to interruption of central autonomic pathways.
- Neurogenic arthropathies (Charcot joints) may affect the shoulder,
elbow, or wrist. Scoliosis is sometimes seen.
- Acute painful enlargement of the shoulder is associated with destruction
of the head of the humerus.
Physical:
- Arm reflexes are diminished early in the clinical course.
- Lower limb spasticity, which may be asymmetrical, appears with other long
tract signs such as paraparesis, hyperreflexia, and extensor plantar
responses.
- Rectal examination includes an evaluation of volitional sphincter control
and sensory assessment of sacral dermatomes.
- There may be dissociated sensory impairment.
- The syrinx may be extend into the brain stem affecting cranial nerves or
cerebellar function.
- Brain stem signs are common in syringomyelia associated with Chiari
malformations.
Causes: Etiology of syringomyelia is often associated with
craniovertebral junction abnormalities.
- Platybasia and basilar invagination
- Assimilation of the atlas
- Soft tissue masses of abnormal nature
- Tumors (eg, meningioma at foramen magnum)
- Cerebellar tonsils and vermis herniation
- Arachnoid cysts, rhombic roof, or vascularized membranes
- Posthemorrhagic or postinflammatory membranes
- Etiology of syringomyelia (not associated with craniovertebral
abnormalities)
- Arachnoid scaring related to spinal trauma
- Arachnoid scaring related to meningeal inflammation
- Arachnoid scaring related to surgical trauma
- Subarachnoid space stenosis due to spinal neoplasm or vascular
malformation
- Subarachnoid space stenosis, with possible scaring, related to disc and
osteophytic disease
 |
DIFFERENTIALS |
Section 4 of 11    |
Acute Inflammatory
Demyelinating Polyradiculoneuropathy
Amyotrophic Lateral Sclerosis
Ankylosing
Spondylitis
Arteriovenous Malformations
Atlantoaxial
Instability in Individuals with Down Syndrome
Central Pontine Myelinolysis
Cervical
Spondylosis: Diagnosis and Management
Chronic Inflammatory
Demyelinating Polyradiculoneuropathy
Diabetic Neuropathy
Hereditary Motor and Sensory
Neuropathies
Hydrocephalus
Limb-Girdle Muscular
Dystrophy
Medulloblastoma
Meningioma
Metastatic Disease to the
Spine and Related Structures
Motor Evoked Potentials
[Motor Nerve Conduction Principles]
Multiple Sclerosis
Neural Tube Defects
Neurological
History and Physical Examination
Spinal Cord Hemorrhage
Spinal Cord
Infarction
Spinal
Epidural Abscess
Spinal Muscular Atrophy
Other Problems to be Considered:
Arnold-Chiari malformations
Cervical rib
Craniovertebral junction
anomalies
Increased intracranial pressure
Intrinsic tumors of the spinal
cord
Brainstem syndromes
Cervical disk syndromes
Lab Studies:
- Cerebral spinal fluid (CSF) analysis
- CSF pressure is sometimes elevated. A complete subarachnoid block may be
noted.
- Cell count is rarely more than 10/mm3.
- Mild elevation of the CSF protein content occurs in half of these
cases.
- In cases of subarachnoid block, CSF protein may exceed 100
mg/dL.
Imaging Studies:
- Cannot detect the syrinx directly.
- Cervical canal commonly is widened and erosion of the pedicles may be
found.
- Flexion and extension films exclude bony instability.
- Basilar impression or craniovertebral anomalies may be
demonstrated.
- Computerized tomography (CT) scan
- Aids in the detailed assessment and is especially useful in the
evaluation of bony spinal canal components.
- Performed in special situations when MRI cannot be used.
- Widening of the cord and complete subarachnoid block may be
observed.
- Myelogram, in combination with immediate and delayed high-resolution CT
scan, can also be performed. Delayed CT scans are obtained 4-24 hours after
the initial testing and can demonstrate cyst filling.
- Magnetic resonance imaging (MRI)
- Imaging of the entire rostrocaudal extension of the cyst or cysts is
important. Add of gadolinium-enhanced images if a tumor is suspected.
Gadolinium-enhanced images are helpful in differentiating between scar or
disc material associated with a syrinx, especially in postoperative or
posttraumatic cases.
- MRI examination should include sagittal and transverse views in T1 and
T2 images. Proton density scans can also be helpful.
- Magnetic resonance angiography
- Can be especially helpful in cases of syringomyelia associated with
vascular lesions.
- MRI routines used to analyze spinal fluid flow dynamics near the spinal
cord cyst.
Other Tests:
- Neurophysiological assessment by somatosensory evoked potentials (SSEPs)-
Low amplitude or delayed responses are present in myelopathy.
- Neurophysiological assessment by motor evoked response may be more
sensitive than SSEPs in the evaluation of spinal cord dysfunction.
Procedures:
- The initial evaluation of patients suspected of having a spinal cord
syrinx includes a comprehensive history and physical examination.
- Information obtained from examinations guides the imaging studies.
Essential tests include plain radiographic series with dynamic views and
high-resolution CT scan to assess the bony spinal canal.
- The most sensitive imaging test for soft tissue is an MRI scan.
Gadolinium-enhanced images are also helpful in differentiating between tumor,
scar, or disc material, especially in postoperative or posttraumatic
cases.
Histologic Findings: The syringomyelic cavity,
or syrinx, forms most commonly in the lower cervical region, particularly at the
base of the posterior horn and extending into the central gray matter and
anterior commissure of the cord.
Histopathological findings include (1) cavitation of spinal cord gray matter,
(2) syrinx continuous with or adjacent to the central canal, and (3) an inner
layer of gliotic tissue.
In association with the syrinx, other pathological conditions such as tumors,
vascular anomalies, or infective processes may also be evident.
| |
TREATMENT |
Section 6 of 11 |
Medical Care:
- No medical management of patients with syringomyelia exists. However, a
chronic stable clinical course is common. It is very important to identify the
underlying cause of syrinx formation. Surgical treatment will most likely be
necessary.
- Neurorehabilitative care facilitates preservation of remaining
neurological functions and prevents complications of quadriparesis such as
infection and decubitus ulcers.
Surgical Care: A variety of surgical treatments have been
proposed for syringomyelia.
- 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.
- Ventriculoperitoneal shunt is indicated if there is ventriculomegaly and
increased intracranial pressure.
- Lumboperitoneal shunt is infrequently placed because of increased risk of
herniation through the foramen magnum.
- Percutaneous needling 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. Morever, it seems unlikely that a
needle track would remain open.
- Syringosubarachnoid dorsal root entry zone shunt
- 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
A fibro-scope inserted through a small myelotomy allows inspection of the
intramedullary cavity. This technique is particularly useful in evaluating and
treating multiple septated syrinxes. Septa are fenestrated, either
mechanically or by laser. Fluid from the cavity is then shunted into the
subarachnoid space.
Consultations:
Diet: No specific diet for syringomyelia is recommended;
however, normalizing weight is encouraged, especially in patients with
neurological deficits.
Activity: n/a
| |
MEDICATION |
Section 7 of 11 |
There is no specific
medication for treatment of syringomyelia. However, analgesics and muscle
relaxants may be given for symptomatic treatment.
Drug Category: Nonsteroidal anti-inflammatory drugs
(NSAIDs) - NSAIDs are commonly used as analgesics in patients with
syringomyelia. If one class seems to be ineffective after 2-wk trial, a
formulation from another class may be tried. The most commonly used drugs are
ibuprofen, acetylsalicylic acid, naproxen, indomethacin, mefenamic acid, and
piroxicam.
Drug Name
|
Ibuprofen (Ibuprin, Advil, Motrin)-
Obtained from propionic acid derivatives group. Effective inhibitor of
cyclooxygenase, which is responsible for biosynthesis of prostaglandins;
rapidly absorbed after PO administration; half-life in plasma is about 2
h; passes slowly into synovial spaces and may remain there in higher
concentration as concentrations in plasma decline; excretion is rapid and
complete, mainly in urine as metabolites or their conjugates.
|
| Adult Dose |
Maintenance dose: 1200-1800 mg PO q4-6h;
not to exceed 3200 mg in divided doses
|
| Pediatric Dose |
Not established
|
| Contraindications |
Documented hypersensitivity to ibuprofen,
other NSAIDs, or aspirin; avoid in peptic ulcer disease, recent GI
bleeding or perforation, renal insufficiency, and high risk of bleeding
|
| Interactions |
Coadministration with aspirin increases
risk of inducing serious NSAID-related side effects; probenecid may
increase concentrations and, possibly, toxicity of NSAIDs; may decrease
effect of hydralazine, captopril, and beta-blockers; may decrease diuretic
effects of furosemide and thiazides; monitor PT closely (instruct patients
to watch for signs of bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased when administered concurrently
|
| Pregnancy |
D - Unsafe in pregnancy
|
| Precautions |
Category D in third trimester of
pregnancy; caution in congestive heart failure, hypertension, and
decreased renal and hepatic function; caution in anticoagulation
abnormalities or during anticoagulant therapy |
Drug Name
|
Aspirin (Anacin, Ascriptin, Bayer
Aspirin)- Treats mild to moderate pain and headache. inhibits
prostaglandin synthesis, which prevents formation of platelet-aggregating
thromboxane A2; acts on heat-regulating center of hypothalamus and
vasodilates peripheral vessels to reduce fever.
|
| Adult Dose |
325-650 mg PO q4-6h; not to exceed 4 g/d
|
| Pediatric Dose |
10-15 mg/kg/dose PO q4-6h; not to exceed
60-80 mg/kg/d
|
| Contraindications |
Documented hypersensitivity; liver
damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders,
asthma; due to association of aspirin with Reye syndrome, do not use in
children (<16 y) with flu
|
| Interactions |
Effects may decrease with antacids and
urinary alkalinizers; corticosteroids decrease salicylate serum levels;
additive hypoprothrombinemic effects and increased bleeding time may occur
with coadministration of anticoagulants; may antagonize uricosuric effects
of probenecid and increase toxicity of phenytoin and valproic acid; doses
>2 g/d may potentiate glucose lowering effect of sulfonylurea drugs
|
| Pregnancy |
D - Unsafe in pregnancy
|
| Precautions |
May cause transient decrease in renal
function and aggravate chronic kidney disease; avoid use in patients with
severe anemia, with history of blood coagulation defects, or taking
anticoagulants |
Drug Name
|
Naproxen (Naprelan, Naprosyn, Aleve,
Anaprox)- For relief of mild-to-moderate pain; inhibits inflammatory
reactions and pain by decreasing activity of cyclooxygenase, which is
responsible for prostaglandin synthesis.
|
| Adult Dose |
500 mg PO, followed by 250 mg q6-8h; not
to exceed 1.25 g/d
|
| Pediatric Dose |
<2 years: Not established
>2
years: 2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
|
| Contraindications |
Documented hypersensitivity; peptic ulcer
disease; recent GI bleeding or perforation; renal insufficiency
|
| Interactions |
Coadministration with aspirin increases
risk of inducing serious NSAID-related side effects; probenecid may
increase concentrations and, possibly, toxicity of NSAIDs; may decrease
effect of hydralazine, captopril, and beta-blockers; may decrease diuretic
effects of furosemide and thiazides; monitor PT closely (instruct patients
to watch for signs of bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased when administered concurrently
|
| Pregnancy |
B - Usually safe but benefits must
outweigh the risks.
|
| Precautions |
Category D in third trimester of
pregnancy; acute renal insufficiency, interstitial nephritis,
hyperkalemia, hyponatremia, and renal papillary necrosis may occur;
patients with preexisting renal disease or compromised renal perfusion
risk acute renal failure; leukopenia occurs rarely, is transient, and
usually returns to normal during therapy; persistent leukopenia,
granulocytopenia, or thrombocytopenia warrants further evaluation and may
require discontinuation of drug |
Drug Name
|
Indomethacin (Indocin, Indochron E-R)-
Rapidly absorbed; metabolism occurs in liver by demethylation,
deacetylation, and glucuronide conjugation; inhibits prostaglandin
synthesis.
|
| Adult Dose |
25-50 mg IR PO bid/tid
75 mg PO SR PO
bid; not to exceed 200 mg/d
|
| Pediatric Dose |
1-2 mg/kg/d PO divided bid/qid; not to
exceed 4 mg/kg/d or 150-200 mg/d
|
| Contraindications |
Documented hypersensitivity; GI bleeding
or renal insufficiency
|
| Interactions |
Coadministration with aspirin increases
risk of inducing serious NSAID-related side effects; probenecid may
increase concentrations and, possibly, toxicity of NSAIDs; may decrease
effect of hydralazine, captopril, and beta-blockers; may decrease diuretic
effects of furosemide and thiazides; monitor PT closely (instruct patients
to watch for signs of bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased when administered concurrently
|
| Pregnancy |
B - Usually safe but benefits must
outweigh the risks.
|
| Precautions |
Category D in third trimester of
pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia,
interstitial nephritis, and renal papillary necrosis may occur; increases
risk of acute renal failure in patients with preexisting renal disease or
compromised renal perfusion; reversible leukopenia may occur, (discontinue
if there is persistent leukopenia, granulocytopenia, or
thrombocytopenia)
|
Drug Name
|
Piroxicam (Feldene)- Decreases activity
of cyclooxygenase, which in turn inhibits prostaglandin synthesis. These
effects decrease formation of inflammatory mediators.
|
| Adult Dose |
10-20 mg/d PO qd
|
| Pediatric Dose |
0.2-0.3 mg/kg/d PO qd; not to exceed 15
mg/d
|
| Contraindications |
Documented hypersensitivity; active GI
bleeding
|
| Interactions |
Coadministration with aspirin increases
risk of inducing serious NSAID-related side effects; probenecid may
increase concentrations and, possibly, toxicity of NSAIDs; may decrease
effect of hydralazine, captopril, and beta-blockers; may decrease diuretic
effects of furosemide and thiazides; monitor PT closely (instruct patients
to watch for signs of bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased when administered concurrently
|
| Pregnancy |
B - Usually safe but benefits must
outweigh the risks.
|
| Precautions |
Category D in third trimester of
pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia,
interstitial nephritis, and renal papillary necrosis may occur; increases
risk of acute renal failure in patients with preexisting renal disease or
compromised renal perfusion; reversible leukopenia may occur, (discontinue
if there is persistent leukopenia, granulocytopenia, or
thrombocytopenia)
|
Drug Name
|
Mefenamic acid (Ponstel)- Inhibits
inflammatory reactions and pain by decreasing prostaglandin synthesis.
|
| Adult Dose |
500 mg initially followed by 250 mg q4h
prn
|
| Pediatric Dose |
<12 years: Not established >12
years: Administer as in adults
|
| Contraindications |
Documented hypersensitivity; peptic ulcer
disease, recent GI bleeding or perforation, renal insufficiency, and high
risk of bleeding
|
| Interactions |
Coadministration with aspirin increases
risk of inducing serious NSAID-related side effects; probenecid may
increase concentrations and, possibly, toxicity of NSAIDs; may decrease
effect of hydralazine, captopril, and beta-blockers; may decrease diuretic
effects of furosemide and thiazides; monitor PT closely (instruct patients
to watch for signs of bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased when administered concurrently
|
| Pregnancy |
C - Safety for use during pregnancy has
not been established.
|
| Precautions |
Category D in third trimester of
pregnancy; may have adverse effects in fetus; caution in congestive heart
failure, hypertension, and decreased renal and hepatic function; caution
in anticoagulation abnormalities or during anticoagulant
therapy |
Drug Category: Muscle
relaxants - Treat muscle spasms to decrease patient's level of
discomfort.
Drug Name
|
Methocarbamol (Robaxin)- Skeletal muscle
relaxant used in conjunction with other therapeutic efforts to treat pain
and discomfort associated with musculoskeletal conditions. Acts on the CNS
to relax certain reflexes.
|
| Adult Dose |
<60 years: 1.5 g PO qid for first
48-72 h; usual maintenance dose is 750 mg to 1 g PO qid or 1.5 g tid, not
to exceed 6 g/d for first 2-3 d or 8 g/d in severe conditions
>60
years: 6 g/d PO initially (8 g in severe cases); reduce dose prn
|
| Pediatric Dose |
>12 years: 800 mg (2 tab) PO
qid
<12 years: Not established
|
| Contraindications |
Documented hypersensitivity; renal
impairment
|
| Interactions |
Increases toxicity of CNS depressants
|
| Pregnancy |
C - Safety for use during pregnancy has
not been established.
|
| Precautions |
Caution in patients with history of
seizures
Side effects include lightheadedness, blurred vision,
dizziness, drowsiness, itching, conjunctivitis, fever, headache, hives,
nasal congestion, nausea and vomiting, rash, urticaria (an itching attack,
may be due to drug sensitivity), anaphylaxis (severe allergic reaction),
extreme weakness, temporary vision loss, transient paralysis
Overdosage
symptoms include convulsions, vomiting, diarrhea, headache, nausea,
difficult breathing, sensation of paralysis, coma, severe weakness
Drug may cause color interference in certain screening tests for
5-hydroxyindoleacetic acid (5-HIAA) and vanillylmandelic acid (VMA)
To
prevent additive CNS depression, avoid drinking alcoholic beverages or
taking other CNS depressants (excessive sleepiness, slurred speech,
decreased awareness)
Observe extreme caution in patients with impaired
liver or kidney function
Patients >60 years, more likely to
experience adverse reactions
Due to risk of potential harm to newborn,
avoid using while breast-feeding
Prolonged use requires regular
monitoring |
| |
FOLLOW-UP |
Section 8 of 11 |
Further Inpatient Care:
- Generally, patients with uncomplicated syringomyelia who have mild,
relatively stable, disability may be followed on an outpatient basis. Patients
with severe disability are better served in the hospital.
- Check for CSF leakage from tubes exiting the dura.
- Provide neck collar for patients, as needed for comfort.
- Reported postoperative complications include the following:
- Worsening of neurological deficit
- Shunt infection or obstruction
- MRI recommended during the early postoperative period, as a baseline for
further studies
Further Outpatient Care:
- The following is documented with each return visit:
- Healing of the surgical incision
- New neurological deficits
- Status of the integument, genitourinary, gastrointestinal, vascular, and
respiratory systems
- Nutrition, affect/mood, activities of daily living, overall disability,
and employment potential
- Urinalysis and assessment of renal function
- Physical therapy
Occupational therapy-An occupational therapist can assist with specific
home or work station modifications. Early referral is indicated to minimize
further immobility or inactivity.
Review by social services, psychologist, recreational therapist,
orthopedist, neurologist or neurosurgeon, urologist, or internist, as
appropriate.
In/Out Patient Meds:
- Nonsteroidal anti-inflammatory drugs (eg, acetylsalicylic acid, naproxen,
ibuprofen, indomethacin, mefenamic acid, piroxicam).
- Muscle relaxants (eg, cyclobenzaprine, methocarbamol, baclofen).
Transfer:
Deterrence/Prevention:
Complications:
- Complications due to myelopathy include the following:
- Paraplegia or quadriplegia
- Bowel and urinary dysfunction
Prognosis:
- Prognosis depends on the underlying cause, the magnitude of neurologic
dysfunction, and the location and extension of the syrinx.
- Patients presenting with moderate or severe neurological deficits fair
much worse than those patients with mild deficits. Patients with central cord
syndrome have poor response to treatment.
- Natural history of syringomyelia is still not well understood. In one
study, one half of the patients were clinically stable for several years and
about 20% of all patients died at an average age of 47 years. Early
intervention may improve the outcome.
- Myelopathy is the most serious consequence of syringomyelia. The following
are 6 grades of disability from myelopathy:
- Grade 0- Root signs and symptoms; no evidence of cord
involvement
- Grade I- Signs of cord involvement; normal gait
- Grade II- Mild gait involvement; employable
- Grade III- Gait abnormality prevents employment
- Grade IV- Ambulates only with assistance
- Grade V- Chair bound or bedridden
Patient Education:
- Avoid high-impact exercise, such as running and jumping in cases
associated with cervical instability.
- Avoid activities involving Valsalva maneuvers.
| |
MISCELLANEOUS |
Section 9 of 11 |
Medical/Legal Pitfalls:
- Overuse of muscle relaxants
- Overuse of pain medication
- Prolonged rest or inactivity
- Failure to recognize chronic pain syndrome
- Surgical complication of infection and spinal cord trauma
Special Concerns:
| |
PICTURES |
Section 10 of 11 |
| |
BIBLIOGRAPHY |
Section 11 of 11  |
- Boman K, Livanianen M: Prognosis of syringomyelia. acta neurol scand 1967;
43: 61-68.
- Colombo A, Cislaghi MG: Familial syringomyelia: case report and review of
the literature. Ital J Neurol Sci 1993 Dec; 14(9): 637-9[Medline].
- Gardner WJ: Hydrodynamic mechanism of syringomyelia: its relationship to
myelocele. J Neurol Neurosurg Psychiatry 1965; 28: 247-259.
- Gruber DP, Crone KR: Neuroendoscopy. In, Grossman RG, and Loftus CM (eds):
Principles of Neurosurgery. Lippincott-Raven 1998; 2nd ed: 757-762.
- Hopkins A: Clinical neurology: A modern approach. Oxford University Press
1993; 342-344.
- Huewel N, Parnecky A, Urban V: Neuroendoscopic technique for the operative
treatment of septated syringomyelia. Acta Neurochir Suppl 1992; 54: 59-62.
- Madsen III PW,, Green BA, Bowen BC: Syringomyelia. In: Herkowitz HN,
Garfin SR, Balderston RA et al.(eds): The Soine. W.B Saunders Company 1999;
4th ed, Vol. 2: 1431-1459.
- Mancall EL: Syringomyelia. In: Rowland LP (ed). Merritt's Textbook of
Neurology. Lea & Febiger 1989; 8th ed: 687-691.
- Milhorat TH, Kotzen RM, Mu HT: Dysesthetic pain in patients with
syringomyelia. Neurosurgery 1996 May; 38(5): 940-6; discussion 946-7[Medline].
- Milhorat TH, Capocelli AL Jr, Kotzen RM: Intramedullary pressure in
syringomyelia: clinical and pathophysiological correlates of syrinx distension
[see comments]. Neurosurgery 1997 Nov; 41(5): 1102-10[Medline].
- Oakes WJ: Chiari Malformation and Syringomyelia. In: Rengachary SS and
Wilkins RH (eds): Principles of Neurosurgery. Wolfe Mosby. 1995; 9.1-9.17.
- Oldfield E, Murasko K, Shawker TH: Pathophysiology of syringomyelia
associated with Chiari I malformation of the cerebellar tonsils. Implications
for diagnosis and treatment. J Neurosurg 1994; 80:(1): 3-15.
- Rhoton AL, Hamilton AJ: Chiari Malformation and Syringomyelia. In: Benzel
EC (ed): Spine surgery: techniques, complication avoidance, and management.
Churchill Livingstone 1999; Vol.2: 793-812.
- Simon RP, Aminoff MJ, Greenberg DA: Clinical Neurology. Appleton &
Lange 1999; 4th ed: 220-221.
- Williams B: A critical appraisal of posterior fossa surgery for
communicating syringomyelia. Brain 1978 Jun; 101(2): 223-50[Medline].
- Williams B: Progress in syringomyelia. Neurol Res 1986 Sep; DA -
19861218(3): 130-45[Medline].
- Wisoff JH, Epstein F: Management of hydromyelia. Neurosurgery 1989 Oct;
25(4): 562-71[Medline].
- Wisoff JH: Chiari Malformations and Hydromyelia. In: Tindall GT, Cooper
PR, and Barrow Daniel (eds): The practice of Neurosurgery. William &
Wilkins 1995; Vol. 3: 2743-2753.
| NOTE:
|
| Medicine is a constantly changing
science and not all therapies are clearly established. New research
changes drug and treatment therapies daily. The authors, editors, and
publisher of this journal have used their best efforts to provide
information that is up-to-date and accurate and is generally accepted
within medical standards at the time of publication. However, as medical
science is constantly changing and human error is always possible,
the authors, editors, and publisher or any other party involved with the
publication of this article do not warrant the information in this article
is accurate or complete, nor are they responsible for omissions or errors
in the article or for the results of using this information. The reader
should confirm the information in this article from other sources prior to
use. In particular, all drug doses, indications, and contraindications
should be confirmed in the package insert. FULL DISCLAIMER
|
Topic last updated March
01, 2001.
© Copyright 2001, eMedicine.com, Inc.
eMedicine
Zoom View (Interactive!)