spontaneous intramedullary hemorrhage is a rare disease entity , and most cases have specific etiologies , such as vascular malformations and tumorous lesions61112 ) .
there are also reports indicating that anticoagulation therapy and hemophilia cause spontaneous intramedullary hemorrhage1313 ) .
it is extremely rare for this to occur without an apparent underlying etiology , and only a few such cases have been reported468910 ) .
recurrent hemorrhage is thought to be the cause of progressive and stepwise neurological deterioration in vascular malformations of the spinal cord2 ) , and a high risk of rebleeding from a vascular malformation is the reason why total excision of the pathology is important .
although there have been some reports suggesting the occurrence of recurrent bleeding in chronic forms of intramedullary hemorrhage without apparent etiology710 ) , it is extremely rare for recurrent intramedullary hemorrhage to occur acutely without a specific cause . here ,
while we describe this rare event , we discuss its etiology , and the diagnostic , therapeutic and prognostic implications of this disease including a review of the relevant literature .
a 48-year - old woman visited our emergency department complaining of voiding difficulty , weakness and sensory disturbances in both lower extremities , which had started 7 days ago prior to her visit .
an initial neurological examination revealed grade iii motor power for the right and grade iv motor power for left lower extremity , and she was unable to walk .
she had been taking an antihypertensive medication for 4 years . otherwise she had no known diseases such as blood dyscrasias , bleeding disorders , cardiovascular disorders , and various other central nervous system disorders .
thoraco - lumbar spine magnetic resonance imaging ( mri ) revealed an intramedullary mass lesion at the t9 - 10 level , which showed as a high signal in a t1 weighted image ( fig .
following gadolinium injection , subtle enhancement at the periphery of the mass was observed ( fig .
midline myelotomy along the dorsal columns was performed , and a dark - colored hematoma was evacuated .
it was mostly liquefied , but some of it was not . keeping cavernous malformation in mind as a possible diagnosis , the dark blood clots were carefully dissected from the spinal cord and sent for histopathological examination . on careful inspection of the intraspinal cavity , neither abnormal vessels nor tumorous lesions
postoperative mri revealed most of the lesion was removed , and no abnormal findings other than postoperative changes were observed ( fig .
an mri was immediately taken , and it showed an increased extent of intramedullary hemorrhage at the operation site ( fig .
laboratory examination revealed no abnormality of coagulation profile ( prothrombin time international normalized ratio , 0.99 ( reference 0.85 - 1.3 ) , activated partial thromboplastin time , 30.7 seconds ( reference 30 - 47 seconds ) ) .
however , we failed to identify any abnormal vasculature or tumorous lesion that might have caused rebleeding .
lesions which might have caused the recurrent bleeding were dissected and biopsied for pathologic diagnosis . during the operation ,
the spinal cord was so severely swollen that we had to perform expansile duroplasty using artificial dura .
the pathology report again confirmed only hematoma , and no abnormal vessels or atypical cells were identified .
a mri that was taken seven days after the second surgery showed a markedly decreased extent of the intramedullary hemorrhage and a spinal cord herniation to the dorsal side , which was due to the expansile duroplasty .
. a rehabilitation program was arranged , and motor power in both of her lower extremities gradually improved .
three months after surgery , motor power in both right and left legs were improved to grade 2 and 3 , respectively . at a 9-month follow - up
in addition , self - voiding became possible with a maneuver to increase abdominal pressure .
what makes this case unique from others is that two confirmed events of intramedullary hemorrhage without specific cause occurred , and that the second event happened a few days after the surgery to perform hematoma evacuation with meticulous bleeding control .
evidence for recurrent bleeding in chronic forms of spontaneous intramedullary hemorrhage are present in the literature10 ) , but it was difficult to find a case similar to the present one .
variable causes of intramedullary hemorrhage , such as vascular malformations , tumorous conditions , and conditions causing coagulopathies and trauma have been proposed4 ) .
although it is extremely rare , there are a few reports of cases of intramedullary hemorrhage without an identifiable etiology .
the stages and extent of hematoma can be seen clearly , and sometimes the underlying pathology can be identified .
the hematoma usually compresses the surrounding neural tissue and the possibility that small obliterated lesions are present that do not appear on a mri scan should be considered . in the present case ,
the periphery of the hematoma was likely to enhance , and our preoperative diagnosis was intramedullary ependymoma with hemorrhage .
however , intraoperative inspection and the pathology report did not find any pathology that might cause hemorrhage .
five days after the initial surgery , rebleeding at the operation site occurred . because it was possible that the etiology had been missed during the first surgery , possible lesions that might cause the recurrent bleeding were dissected and biopsied during the second surgery .
again , no abnormal lesions other than hematoma were confirmed in the final pathology report .
postoperative mris taken after the first and second operations also showed no abnormal enhancement of vessels or mass lesions , except for normal postoperative changes . with these findings , we concluded that the diagnosis of our case was spontaneous idiopathic intramedullary hemorrhage .
some authors disagree with its use if a mri did not show any abnormal vessels56 ) .
idiopathic intramedullary hemorrhage is usually diagnosed by surgery when no underlying pathology is found in macro- and microscopy4 ) .
however , most recurrent hemorrhage cases in central nervous system have a vascular pathology with a bleeding focus , and it is logical to think so in our case . in this context , spinal angiography would have been helpful to identify the etiology of recurrent bleeding , and we think that it was necessary to do the angiography on looking back upon this case .
most of the literature recommends early diagnosis and surgery for cases of intramedullary hemorrhage with progressive neurologic deterioration4569 ) .
in addition , our case suggests that delayed hemorrhage can occur without the presence of an etiology even after meticulous bleeding control is performed in a previous surgery .
approximately 9 months later , motor power in both legs improved and the patient could walk a short distance with the aid of a walker .
we emphasize that surgical intervention should be performed as soon as possible to preserve and restore neurological functions in cases of idiopathic spontaneous intramedullary hemorrhage .
because spontaneous intramedullary hemorrhage with unknown etiology is very rare , meticulous inspection of the hemorrhagic site to find the cause is essential .
repeated hemorrhage can occur ; close observation of patients after surgery is therefore important in cases without an apparent etiology .
 a 48-year - old woman presented with acute voiding difficulty , numbness and weakness of both lower extremities . magnetic resonance imaging ( mri ) showed an intramedullary hemorrhagic mass that extended from t9 to t10 . 
 t8-t10 laminotomy and surgical removal of the hemorrhagic mass was performed . 
 the pathological diagnosis was hematoma . 
 her neurological status remained the same after the operation . at 5 days post - operation , 
 the patient suddenly became paraplegic , and mri that was immediately performed revealed a recurrent intramedullary hemorrhage . 
 emergent surgical evacuation was performed . 
 again , histological examination showed only hematoma , without any evidence of abnormal vessels or a tumor . 
 a postoperative mri revealed no abnormal lesions other than those resulting from postoperative changes . at a 9-month follow up , 
 the patient could walk a short distance with the aid of a walker . because spontaneous intramedullary hemorrhage with unknown etiology is very rare 
 , it is essential to perform a meticulous inspection of the hemorrhagic site to find the underlying cause . 
 repeated hemorrhage can occur ; therefore , close observation of patients after surgery is important in cases without an apparent etiology . 
 urgent surgical evacuation is important to improve outcomes in these cases . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION