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433
127.7 Management
• IV methylprednisolone for 5 days, followed by oral hydrocortisone for 7–10
days, gradually tapered
• IM chlorpheniramine
• Plasma exchange
• IV gelofusine in severe hypotension
• subcutaneous adrenaline
Recovery may be partial, subtotal, or incomplete, depending on toxicity, aller-
genecity, duration, and treatment. Thus far no antivenom is available.
Reference
Likittanasombut P, Witoonpanich R, Viranuvatti K. Encephalomyeloradiculopathy associated with
wasp sting. J Neurol Neurosurg Psychiatr. 2003;74(1):134–5.
Reference
Part VIII
Vascular Causes of Myelopathy
437
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_128
128
Anterior Spinal Artery Syndrome
128.1 Definition
Anterior spinal artery syndrome (ASAS) or Spiller’s syndrome is named for William
G. Spiller who described it 1909, however Preobranshenski was the fi rst to describe
ASAS in 1904. ASAS is thrombosis ventral to the spinal cord causing blockage of the
anterior spinal artery (ASA) and an infarction of the areas supplied. Outside the spinal
cord there are some collateral anastomoses, which may help in vascular obliteration.
There are anastomoses between the deep cervical, the vertebral and occipital arteries. In
addition, the ascending cervical and muscular branches anastomose. There are also
anastomoses between the two vertebrals and the radicular arteries at the lower end of the
cord. There are cruciate anastomoses between the artery of Adamkiewicz, the posterolateral arteries and the branch from the caudal vessels in the thoracic region the anastomosis is scanty so that it is a watershed area. Addressing the venous drainage, the veins
that drain the cord correspond to the arteries but they are not exactly alike. The have
seven categories based on their size. The medullary veins drain into the spinal and into
the radicular veins. These anastomose with the epidural veins and the veins of the vertebral bodies and eventually drain into the vertebral, intercostal, lumbar, and sacral, veins.
Ultimately they drain into the jugular, azygos, and hemiazygos veins, thus connecting
with the venous system of the chest, abdominal, and pelvic cavities. This is what Batson
described in the 1940s when he divided the venous plexus from the occiput down to the
sacrum into three groups: extradural, intravertebral, and extravertebral.
128.2 Incidence
Foo and Rossier ( 1983 ) reviewed 256 spinal cord injuries and found only thirteen
(0.05 %) cases of ASAS. Zantl, et al., report ASAS in 0.2 % in 1000 esophagectomies over a 18 year period. The prevalence of these syndromes ranges from 0.5 %
in operations for coarctation of the aorta to as high as 15 % after surgery for thoracoabdominal aneurysms.
438
128.3 Etiology
The spinal cord is due to ischemia, although there are several sources of vascularization. Functionally, the vascularization is not always adequate, since the
ASA is not a continuous channel and the perimedullary do not anastomose with
the intramedullary area. Furthermore, the intramedullary anastomosis has no
functional value. If the blood pressure falls below the critical level, an infarction
occurs. This depends on the site of artery occlusion and the collaterals. It also
depends on the onset of anterior occlusion; a slow onset is more tolerable than
that of the acute. The extent of damage depends on the perfusion pressure; a pressure of 30 mmHg will cause hypoperfusion and hypoxia, both of which aggravate
the damage to the spinal cord. There are anatomic variations, which modify the
ischemic effect.
128.4 Pathology
The result of arterial occlusion is infarction of the spinal cord. This leads to necrosis
of the involved tissue. There may be minor hemorrhage, cellular infi ltration by enormous numbers of scavengers derived from the microglia and the micropahges.
These may be fi lled with fat globules taken up from the disintegrating myelin. The
walls of the vessels around the occlusion may be infi ltrated with microglia. The
peripheral area or the periumbra may have partial recovery depending on the degree
of vascular occlusion and management. Eventually the infracted area will have a
glial scar formed by the astrocytes.
128.5 Clinical Picture
The symptoms of ASAS are usually sudden and manifested within minutes or hours
of the ischemia. There is local or radicular back pain; the nature of the pain may be
burning or lancinating. The pain varies in duration it may only be transient. There is
usually diffused deep aching pain in both legs. Burning dysthetic pain may start in
the feet and ascend up to the abdomen. Soon after the onset of pain there is weakness of the lags and inability to walk, becoming a disability with in minutes. The
syndrome varies according to the level of ASA occlusion. There is loss of pain and
temperature sensation below the level of the lesion; in addition, there is loss of
bowel and bladder control. There is sacral sparing. Partial touch sensation is preserved, as well as proprioception and vibration sense because of the posterior arterial plexus supplying the posterior columns. The leg paralysis is an upper motor
neuron due to a lesion in the lateral corticospinal tract. If ischemia involves only the
sulcal branches there is a lower motor neuron defi cit.
Sometimes signs are restricted to those of either upper or lower motor neurons or
both in a pattern similar to that of amyotrophic lateral sclerosis (ALS). However, the
major differences are mod of onset and the lack of progressive worsening of the
128 Anterior Spinal Artery Syndrome
439
ASAS. Sulcal thrombosis of ASA leads to lower motor neuron defi cit (amyotrophy). This may be unilateral or bilateral depending on the of the sulcal arteries.
There is usually motor weakness without sensory changes. The picture varies
according to the level of ischemia.
The spasticity in the lower limbs is due to the lesion involving the corticospinal
tract and the fl accidity is due to involvement of the gray matter and the anterior
horns.
Transient ischemic attacks (TIA) of the spinal cord and the cauda equine lead to
cord or cauda claudication as describe by Déjérine in 1906. It causes transient weakness and/or numbness of one or both lower extremities during walking and parathesia in one or both feet. If the patient continues walking they might get a drop foot
due to ischemia of the cord; or may develop spastic weakness and increase or exaggerated refl exes if the cord itself is involved. Whereas the weakness due to the cauda
the refl exes will be reduced or absent. The spinal intermittent claudication may
precede the spinal cord infarction caused by ASA thrombosis.
128.6 Diagnosis
The acute onset of ASAS with motor weakness and discriminative anesthesia are
typical of cord infarction. In typical cases, slow onset over time is usually against
ASAS. Atypical cases need to be differentiated from: multiple sclerosis (MS),
ALS, poliomyelitis, cord compression, and transverse myelitis. These can be confi rmed by radiography, myelography, computed tomography scan, and magnetic
resonance imaging. Lumbar puncture may confi rm the diagnosis of MS by showing an elevated gamma globulin in the cerebrospinal fl uid (CSF). In neoplasms
there is an increase of the CSF protein to a very high level. In ASAS there is slight
protein content elevation, but the gamma globulin is normal. Associated infarction
of the vertebral body is sometimes an important sign for diagnosis.
Neurophysiological studies and somatosensory evoked potentials will confi rm spinal cord infarction.
128.7 Management
Treatment should be started very early after the onset of the symptoms two things
must be corrected the hypotension and the hypoxia. The use of anticoagulants and
anti-platelets did not contribute to the improvement of the ischemia. The following
drugs have been tried:
1. Nalaxone hydrochloride
2. Calcium channel blockers
3. Steroid therapy (methylprednisolone)
4. Free radical scavengers
5. Barbiturates
128 .7 M ana gement
440
For the persistent cord edema hyperbaric oxygen may help resolve the edema.
For established paralysis the standard spinal cord rehabilitation programs should be
involved.
Reference
Foo D, Rossier AB. Anterior spinal artery syndrome and its natural history. Paraplegia.
1983;21(1):1–10.
128 Anterior Spinal Artery Syndrome
441
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_129
129
Cervical Myelopathy Caused by Bilateral Persistence of the First Intersegmental Arteries
129.1 Definition
This is a condition of compression myelopathy due to bilateral persistence of the
fi rst intersegmental artery (vertebral artery anomaly).
129.2 Incidence
This is a very rare condition noticed in less than 1 % of angiograms. According to
Takahashi et al., only four cases have been operated.
129.3 Etiology
This is a congenital anomaly in which there is anomalous vertebral arteries (VA) can
cause upper cervical cord compression.
129.4 Pathology
According to Padget, fenestration of the VA is considered to be a persistence of the
intersegmental artery during embryogenesis. Failure of resegmentation of the
embryonic scleratoma seems to promote the abnormal development of the intersegmental arteries. Previous reports show that fenestration of the VA at the craniovertebral junction was often associated with osseous anomalies, such as occipitalization
of the atlas, Klippel-Feil syndrome, hypoplasia of the atlas, basilar impression, cleft
Abstracted from Yamazaki et al. ( 2004 )
442
of C1 posterior arch. The osseous anomalies have been explained as a failure of
rearrangement of the embryogenic scleratomas. The resulting longer intradural
course forms a longer ventral loop, causing cord compression. Other anomalies may
be present e.g. Down’s syndrome.
129.5 Clinical Picture
This anomaly may be asymptomatic and may be detected by angiography for other
reasons. It affects both genders equally. Age ranged from 38 to 75. Symptoms are
usually: cervical pain, occipital neuralgia, accessory nerve palsy, pain in the shoulder and/or the arm. Myelopathy presents with bilateral compression of the dorsal
and dorsolateral columns by the abnormal VAs. The pain may be intermittent due to
VA pulsations on the dorsal root entry zone (DREZ) of the cervical nerve. Later in
life, atheroslecosis, pain hammering style, this may be due to compression of the
Lissauer’s tract, which lies in front of the DREZ. It decussates on or more segments
higher than the DREZ.
129.6 Diagnosis
Diagnosis based on:
1. Confi rmation of clinical picture
2. Laboratory tests 3D computed angiography, which will show the abnormal
course of Vas at C1-C2 level. The course of the VAs is abnormally long intradurally. The arteries form vascular loops reaching the medullar and compressing
the cervical cord.
3. MRI of the spinal cord showed T2 weighted-imges reveals bilateral signal void
areas in the postrolateral areas of the spinal cord at C1
129.7 Management
Suboccipital craniotomy and C1 laminectomy give good approach. With microsurgery the VAs are mobilized laterally to relieve pressure on the cord and are anchored
laterally to the corresponding side of the dura mater with cortex bands. Other techniques may be opted
U p d a t e
Abstract from Ozpinar et al. 2015
Vertebral artery fenestration occurs when the vessel lumen is divided into two
separate channels that eventually fuse, forming the primary vessel. Autopsy and
129 Cervical Myelopathy Caused by Bilateral Persistence
443
angiographic studies suggest that the incidence of vertebral artery fenestration is
0.23–1.95 %. Although fenestration of the vertebral artery can occur either intra- or
extracranially, extracranial fenestration at the upper cervical level is more commonly reported. When the vertebral artery is fenestrated, each channel has its own
muscularis layer and is lined by a separate endothelium. The term fenestration has
been synonymously used with duplication in the literature; however, the two
describe different anatomical phenomena.
Fenestration of the vertebral artery is a rare vascular anomaly that has been
observed at autopsy and on angiography. It is most commonly seen in the extracranial segments of the vertebral artery. This congenital anomaly can arise during multiple different stages of embryological development of the vertebral artery. The
clinical signifi cance is unclear, but multiple studies have reported association with
other vascular anomalies. Awareness of vascular anomalies is crucial to avoid iatrogenic injuries during endovascular diagnostic and therapeutic interventions. Here,
we present a case of a patient with an intracranial vertebral artery fenestration that
was identifi ed during work-up for a foramen magnum mass.
Reference
Ozpinar A, Magill ST, Davies JM, Mcdermott MW. Vertebral Artery Fenestration.
Cureus. 2015;7(1):e245.
Reference
Yamazaki M, Okawa A, Aramomi MA, Hashimoto M, Masaki Y, Koda M. Fenestration of verte-
bral artery at the craniovertebral junction in down syndrome: a case report. Spine.
2004;29(23):E551–4.
Reference
445
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_130
130
Myelopathy in Cobb Syndrome
130.1 Definition
Cobb syndrome is a rare non-inherited clinical entity characterized by the combination of vascular cutaneous nerves and an angioma in the spinal canal at the same
metamere. Cobb described it, in 1895, as a metameric angiomatosis with segmental
involvement of the skin, subcutaneous tissues, vertebral column, dura, and spinal
cord, i.e. a cutaneomeningospinal angiamatosis.
130.2 Incidence
Brant, et al., quoted 29 cases from the literature, in 1999; in 2003, Soeda et al. added
an additional infant case. In 2008, Clark et al. systematically reviewed the literature
and found 39 cases. Statistics cannot be valid since only the symptomatic cases are
reported.
130.3 Etiology
According to Brant et al.:
During fetal development, each somite divides into a medial half which forms the
vertebral bodies (the sclerotome) and the lateral half which develops the muscles
(the myotome). The vertebral bodies create the perichordal tube, and other cells
from the sclerotome migrate dorsally and form the posterior elements. The blood
supply to the vertebrae and spinal cord for the metameres originates from the
Abstracted and reported by Brant et al. ( 1999 ).