Key-words:
Acute - cervical - carotid - dissection - epidural hematoma - magnetic resonance imaging
- sleep - spinal - spontaneous - stroke
Introduction
Acute spontaneous cervical spinal epidural hematoma (ASSEDH) is a well-recognized
but rare condition. Spontaneous spinal epidural hematoma has been reported to be associated
with vascular malformations, tumors, infections, pregnancy, rheumatoid arthritis,
atherosclerosis, and hypertension. The term “spontaneous” has been defined here as
“no identified etiology.”First described by Jackson in 1869,[[1]] 119 cases have thus far been reported, using “spontaneous spinal epidural hematoma
during sleep” as keywords. Out of 119, we found only one English and two Japanese
pieces of literature, indexed in Medline, that reported with a similar presentation
but different etiologies.[[2]],[[3]],[[4]],[[5]],[[6]],[[7]],[[8]],[[9]],[[10]],[[11]],[[12]],[[13]],[[14]],[[15]]
Case Presentation
A 42-year-old army officer with no previous medical illness presented to the emergency
department with acute onset of neck pain that woke him up from sleep, progressing
to left-sided body weakness. He was not on any anticoagulants or traditional medication.
He denied any spinal trauma or prior cervical spondylosis. On admission, he was fully
conscious (Glasgow coma scale 15/15), with stable hemodynamic. He had no contusion
or swelling of the head, neck, or back but was tender to palpation from the level
C3–C6. The pupils were equal and reactive bilaterally. He had normal cranial nerve
function. The motor examination showed muscle strength of (5/5) on the right upper
and lower extremities, whereas the left side had hemiparesis (3/5). The sensory examination
showed pinprick and light touch sensation reduced on upper limb C5/C6/C7-T2/T3 level
on the right side. The left side had normal sensation. Deep tendon reflexes were absent
at the biceps C5/C6 bilaterally, whereas patella and ankle reflexes were normal bilaterally.
Simultaneously, he developed urinary retention (1500 cc) on urinary bag past–urethral
catheterization. The anal sphincter tone was normal. The cerebellar examination seemed
normal given the strength limitation. After obtaining a negative doppler ultrasonography
and cranial CT scan result, an emergent megnetic resonance imaging (MRI) study revealed
a cervical hematoma extending from C3/C4 [[Figure 1]] and [[Figure 2]], mainly on the left side. The patient underwent C3/C6 hemilaminectomy [[Figure 3]], and evacuation of hematoma was carried out within 24 h of presentation. Pathological
examination of the ablated fragments revealed fibrinous and hemorrhagic material,
but there was no histological evidence of tissue. There was a significant improvement
in the weakness on the left side when the patient was discharged from the hospital
at the 12th day of the operation. He had complete functional recovery at the 2 weeks
of follow-up.
Figure 1: (a) The Tl-weighted images showed an isointense, dorsolaterally situated epidural
collection on the left side extending from C4 to C6. (b) The T2-weighted images displayed
a heterogeneous signal within the lesion
Figure 2: Axial T2-weighted scan of the cervical spine revealed a left dorsolateral hypointense
epidural collection with compression of the spinal cord suggestive of acute epidural
hematoma
Figure 3: (a) C4/C6 hemilaminectomy performed, left paramedian localized hematoma. (b) Post
evacuation of hematoma, there is no other abnormality seen under a microscope
Discussion
Spontaneous cervical epidural hematoma was first described in 1869 by Jackson[[1]] and the first surgical approach was performed in 1946 by ver Brugghen.[[3]] Vascular malformation,[[4]],[[15]] anticoagulant therapy,[[5]],[[15]] and neoplasm or systemic disease[[16]],[[2]],[[3]],[[4]],[[5]],[[6]],[[7]],[[8]] are well-known causes. However, the cause of the bleeding remains unknown in 40%
of the cases and these are called an idiopathic spontaneous cervical epidural hematoma.[[5]],[[15]] It is still debatable whether the etiology of spontaneous epidural hematoma is
arterial or venous. Those who support the theory of venous origin claim that epidural
veins have thin walls and no valves. As a result of a sudden increase of intrathoracic
and intra-abdominal pressure after Valsalva maneuvers such as coughing, sneezing,
straining, swimming, defecation, micturition, vomiting and coitus, and lacerations
may occur in the venous plexus causing epidural hemorrhage. However, as seen in our
case, ASSEDH occurred during sleep without any predisposing factor as mentioned earlier,
our patient was at complete rest. Nevertheless, there is some relationship that exists
between sleep and hemodynamic instabilities that can cause end-organ damage such as
morning stroke, acute myocardial infarct, and sudden cardiac death.[[7]] Beatty and Winston analyzed the arterial circulation of the cervical region and
considered the hypothesis that the arterial structures located posteriorly and posterolaterally
in the epidural space were responsible for the hemorrhage.[[6]] ASSEDH generally presents with a sudden onset of acute neck pain. Pain radiation
alters according to the localization of the hematoma on the spinal cord and the nerve
roots. The second most common symptom is the weakness of the limbs, seen below the
compressed spinal cord. Paresis can increase within minutes or days or rarely reoccur.
Spontaneous cervical epidural hematomas need emergent surgical decompression, especially
in cases with neurological deterioration although some cases with spontaneous remission
without any surgical treatment have been reported.[[17]],[[18]],[[19]],[[16]],[[9]] Total laminectomy is the best choice as a surgical approach, but hemilaminectomy
can be preferred according to the location of the hematoma.[[17]],[[18]],[[14]] In our case, the patient with left-sided hemiparesis presented to the emergency
room 6 h after the event. It was initially evaluated as a cerebrovascular accident.
After obtaining a negative result of cranial computed tomography scan, detailed neurological
examination was carried out. Cervical pathology was considered and it was supported
by MRI. The patient was operated within 10 h of his presentation and he has nearly
intact motor neurological examination in the 2nd month of the operation.
Conclusion
Acute spontaneous cervical spinal epidural hematoma is a clinical condition with a
wide variety of initial presentations and is thus easily misdiagnosed. ASCEDH has
a fatal progressive behavior in cases when the diagnosis is delayed. Clinicians should
keep in mind that the clinical presentation of the cervical epidural hematomas can
be acute hemiparesis and it can be misdiagnosed as an ischemic stroke, for which intravenous
thrombolysis is contraindicated in the emergency room. Patients must therefore be
evaluated from this perspective. Surgical decompression must be preferred in patients
presenting with neurological deterioration. However, the author presents a unique
case of ASCEDH that occurred while the patient was asleep with the absence of significant
precipitating factors mentioned in the literature.
Declaration of patient consent
The authors confirmed that all appropriate patient consent forms have obtained. In
the form, the patient has given consent for his images and other clinical information
to be reported in the journal. The patient understands that his names and initials
will not be published and due efforts will be made to conceal their identity.
