spontaneous spinal epidural hematoma ( sseh ) is a very rare cause of spinal cord compression , especially in children .
although sseh has been reported to occur in all age groups , it is most frequent after the fourth or fifth decade16 ) .
sseh is most often defined as a hematoma occurring in the absence of any trauma or iatrogenic procedure3 ) , however , gopalkrishnan et al.7 ) suggest that it should be named when hematoma is of idiopathic origin .
clinical presentations of sseh are miscellaneous and vague , therefore differential diagnosis is important , especially in child . here , we present the first case of sseh concomitant with gbs in a child .
a previously healthy 32-month - old male presented to the emergency department ( ed ) with a 1-week history of progressive paraparesis .
the patient had a cough and fever for about 2 weeks prior to admission to pusan national university yangsan hospital .
five days before admission , he had difficulty moving his legs spontaneously , 4 days before admission he could not walk without mother 's help . on initial evaluation in the ed ,
his neurologic examination was significant for 1/5 strength in his both leg and deep tendon reflexes were absent .
lumbar puncture was performed and cerebrospinal fluid ( csf ) examination revealed increased level of protein ( 544mg / dl ) without any other abnormality .
electromyography showed decreased conduction velocity and amplitude on lower limb . under diagnosis of guillain - barr syndrome ( gbs )
, he admitted to the pediatric department and received intravenous immunoglobulin ( ivig ) therapy .
strength in his both leg was gradually improved ( 3/5 ) after four days of ivig therapy , but he suddenly started to complain about neck pain .
spinal magnetic resonance image ( mri ) demonstrated a dorsal epidural hematoma extending from c4 to t3 level with cord compression ( fig .
1 ) . conventional spinal angiography was performed to rule out spinal vascular abnormality and it revealed negative finding .
his neurologic symptoms were neither improved nor progressed , and the patient was under febrile condition because of bronchopneumonia .
follow - up spinal mri with contrast demonstrated enhancement of spinal nerve roots consistent with gbs and there was no significant interval change of the epidural hematoma ( fig .
2 ) . c7 and t1 left hemilaminectomy and drainage of epidural hematoma was performed after 2 weeks of diagnosis of spinal epidural hematoma .
xanthochromic fluid was expelled and thecal sac expansion was identified in the surgical field . the postoperative course was uneventful and the patient regained strength , making a full recovery during 1 week of follow - up .
sseh is a rare surgical emergency , comprising less than 1% of spinal space - occupying lesions18 ) . since the first case of sseh was reported by jackson in 1869 ,
although sseh has been reported to occur in all age groups , it is most frequent after the fourth or fifth decade , but is rare in children816 ) .
clinical presentations of sseh are usually nonspecific and depend on the involved vertebral level , therefore the diagnosis of sseh is difficult and differential diagnosis is important .
there are several reports of sseh mimicking meningitis , subarachnoid hemorrhage , gbs and even stroke5101315 ) .
although paraparesis or tetraparesis is a common symptom of cervical sseh , hemiparesis can also be presented when the hematoma compresses the unilateral corticospinal tract .
the sseh located at the cervicothoracic junction , as in this case , may be presented with progressive paraplegia and neck pain .
cakir et al.5 ) described a case of sseh mimicking gbs in a child . the most common pattern of gbs is the ascending paralysis , weakness beginning in the lower limbs first and migrating towards the trunk , and this is analogous to the symptoms of the sseh located at the cervicothoracic junction .
however , in the present case , there are several evidences of gbs ; ( 1 ) albuminocytologic dissociation at csf analysis , ( 2 ) decreased conduction velocity and amplitude on electromyography and ( 3 ) nerve root enhancement on enhanced spinal mri . to the best of our knowledge ,
the diagnosis of gbs is mainly based on clinical features and electrophysiologic and csf studies .
however , there are several reports of that enhanced spinal mr imaging can be used as a supplementary diagnostic modality in the diagnosis of gbs , when the clinical and electrophysiologic findings are equivocal12 )
. the presence of nerve root enhancement on enhanced spinal mri can be the evidence of gbs , in the present case . because nerve root generally does not take up much gadolinium on enhanced mri , nerve root enhancement is thought to be secondary to a breakdown of the blood - nerve barrier .
although gbs is a potentially life - threatening disease , even with the best treatment available , there are several reports of good prognosis in children , especially1217 ) .
salehiomran et al.17 ) reported that 16 of 17 patients recovered completely ( 94.1% ) .
immediate administration of ivig after clinical suspicion is recommended , before irreversible nerve damage has taken19 ) . even though the pathogenesis of sseh is
the vertebral venous plexus , a valveless low pressure system , is in continuity with the abdominal and thoracic venous system .
lack of valves in the epidural venous plexus makes the area especially vulnerable to any intrinsic change in pressure .
the bleeding can occur after activities such as voiding , sneezing , bending , coughing , and coitus .
such activities of normal living can cause fluctuations in the intrathoracic and intra - abdominal pressures , with may cause reversal of blood flow resulting in rupture of a delicate vein in the valveless epidural plexus16 ) . in the present case , repeated cough associated with upper respiratory tract infectio ( uri ) may be the cause of sseh result from fluctuations of pressure in abdominal and thoracic venous system , as mentioned previously . however , beatty and winston3 ) suggest that the origin of sseh is arterial origin , because the pressure of the epidural venous plexus is lower than the intrathecal pressure , hematomas usually form quickly , and arterial hypertension may sometimes coexist .
some suggest that these arteries are susceptible to abnormal stretching during symmetrical arm movements3 ) .
sseh can have catastrophic consequences including permanent neurologic deficit and even death , therefore urgent surgical decompression should be considered .
nevertheless , there have been a very few cases in which a good outcome after conservative mannagement4611 ) .
preoperative neurologic deficit and the interval between symptom onset and surgical decompression are believed to be associated with the neurological outcome714 ) .
conservative management and delayed surgical decompression may be justified when there are minimal neurological findings or evidence of an early resolution of neurological deficits , as presented case .
sseh is rare neurosurgical emergencies and require immediate diagnosis and treatment , because it can cause catastrophic consequences .
however , clinical manifestations of the sseh are nonspecific , so it seems to be misdiagnosed such as gbs , meningitis , and stroke . as seen in this case , sseh can be concomitant with gbs and performing urgent spinal mri should be considered when the patient has symptoms like neck pain and meningeal irritation sign .
 spontaneous spinal epidural hematoma ( sseh ) has been reported as a rare cause of spinal cord compression , especially in children . 
 clinical features are usually nonspecific , although cervicothoracic location of hematoma could be presented with progressive paraplegia . 
 guillian - barr syndrome ( gbs ) is clinically defined as an acute peripheral neuropathy causing progressive limb weakness . 
 because sseh and gbs have very similar signs and symptoms , sseh could be misdiagnosed as gbs . 
 nevertheless , they can be presented together . 
 we describe a rare case of sseh coexisting with gbs . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION