the aim of this case report was to study that headache maybe the initial symptom in rasmussen 's syndrome ( rs ) . we studied a case of rs in which the patient experienced a recurring headache for about one year prior to the onset of partial seizures . magnetic resonance imaging ( mri ) results were normal when the headache first occurred and showed left brain atrophy three years later . it was difficult to relieve the patient 's headache , even once seizures were controlled . rasmussen syndrome ( rs ) , also called rasmussen 's encephalitis , is a chronic t - cell - mediated disorder that usually affects one cerebral hemisphere , and that typically presents in childhood . in 2005 , a panel of experts established formal rs diagnostic criteria . the nonspecific prodromal period lasts for about 7  months ( range : 0  months to 8  years ) and may involve mild signs and symptoms , typically including low frequent focal onset seizures and rarely mild hemiparesis . in the subsequent acute phase , which lasts for about 8  months ( range , 48  months ) , frequent focal onset seizures occur , sometimes presenting with epilepsia partialis continua ( epc ) , accompanied by progressive cortical dysfunction . finally , patients in the residual stage exhibit stable neurologic deficits and continuation of seizures . however , some cases involve atypical features , manifesting absence or delayed - onset seizures , and dual pathology , suggesting that focal onset seizures are not necessary for rs diagnosis . we reported a case of rs in a child who presented with the initial onset of headache , and who later was admitted to the hospital several times with the chief complaint of headache , even after her seizures were controlled . a nine - year - old girl with normal development presented with a headache and a feeling like her whole head was expanded without localizing characteristics , unaccompanied by nausea or vomiting , and having no obvious etiology about five years ago . the headache was mild and could remit spontaneously , but repeat about five times , each time with a duration of about 15  days . one year after the onset of headache , this patient experienced a headache that lasted for several hours , with a 2-minute generalized tonic after this incident , the patient frequently experienced focal motor seizures involving the right lower limb without losing consciousness . electroencephalograph ( eeg ) confirmed the presence of epileptiform discharge with increased slow waves in the whole brain without focal abnormalities . epilepsy was diagnosed , and levetiracetam ( about 20  mg / kg / d , twice a day ) was prescribed . the patient 's symptoms showed no improvement after taking oral levetiracetam for about two months . at this time brain mri revealed abnormal signal foci in the left frontal cortex , and eeg showed diffuse slowing with epileptiform discharge in the left hemisphere . lumbar puncture revealed normal cerebral spinal fluid ( csf ) cell count , protein , glucose , chloride , and cryptococcal meningitides . blood test results were normal for ceruloplasmin , lactic acid , ammonia , immunoglobulins ( iga , igm , igg ) , rheumatoid factor ( rf ) , antistreptolysin o ( aso ) , c - reactive protein , autoantibody to nuclear antigen ( ana ) , extractable nuclear antigen ( ena ) , and autoantibody to double - stranded dna ( anti - dsdna ) were negative . rasmussen syndrome ( rs ) was diagnosed according to the diagnostic criteria ( part a ) . the patient received iv methylprednisolone ( 25  mg / kg / d ) pulse therapy for three days and intravenous immunoglobin ( ivig , 400  mg / kg / d ) for five days . the patient also received oral prednisone ( 1  mg / kg / d , three times a day ) , carbamazepine ( 10  mg / kg / d , twice a day ) , and phenobarbital ( 10  mg / kg / d , three times a day ) for seizures , and oxycodone  acetaminophen ( oxycodone 5  mg with acetaminophen 325  mg per tablet , one tablet three times a day as needed ) for headache . after about five months , the seizures stopped , and the patient 's parents discontinued the patient 's antiseizure medication without consent from the doctors . after approximately half a year without headache and focal seizures , the headache and focal seizures resumed . occasional skin rash developed when the patient was treated with carbamazepine , and this was a reason for the discontinuation of treatment . the patient was then prescribed sodium valproate sustained release tablets ( 20  mg / kg / d ) and oxycodone  acetaminophen ( one tablet three times a day as needed ) , with follow - up every three months , and with blood concentration of sodium valproate maintained at around 90  g / ml . 1a ) showed atrophy of the left frontal temporal lobe and deep gray matter , large patchy hypointensities on t1 and increased t2 signals , and bilateral ventricular enlargement . the headache disappeared and the patient was seizure - free for about 2  years and 6  months , at which time the treatment with sodium valproate and oxycodone  acetaminophen was stopped . after about 6  months without symptoms , the headache resumed , and the patient was hospitalized again . the headache was mild to moderate and could be tolerable , and only localized in the left unilateral head region . at this time 1b ) revealed atrophy of the left cerebral hemisphere , with this space occupied by abnormal t1 and t2 signals , as well as enlargement of the third and fourth ventricles and the left ventricle . physical examination showed clear consciousness ; dysarthria ; defects in calculation ability ; weakness of the right lower limb ; decreased grip strength of the right hand ; slightly enhanced muscular tone of the right limbs ; and normal movement , strength , and muscular tone in the left limbs . the patient 's right babinski sign was positive , and the left one was negative . a consulted neurosurgeon advised that the patient was not an optimal candidate for surgical treatment . because of concerns regarding side effects of corticosteroids , ivig ( 400  mg / kg / d ) was administered for 5  days with the informed consent of her parents . the headaches eased , but dyskinesia in the right limbs remained , and oral oxycodone in rs , the initial damage to the brain is mediated by t cells and microglia , and there may be a window for treatment if rs is diagnosed early enough . inflammatory processes observable with mri can support an rs diagnosis , and early diagnosis may be easier in typical cases that closely align with the diagnostic criteria . on the other hand , diagnosis remains challenging in some atypical cases , as there are not yet diagnostic biomarkers available . in our present case , the patient suffered from headaches for about one year prior to her first focal seizure . headaches without functional loss are very common among children and are often due to migraines in the absence of abnormalities upon ancillary testing . thus , a diagnosis of rs was not suspected in our case during the first year . we can not confirm that the headaches in our case were specifically symptoms of rs , but some pathogenic factors for rs may also stimulate headaches . it is possible that some cases of rs may have the same pathogenesis as some headaches , and that analyzing headaches in rs may represent a new route to clarifying rs pathogenesis . the headache could appear and disappear without treatment at the start , and then became a concomitant symptom when the patient was diagnosed with epilepsy . when the headache relapsed the third time , it was hard to tolerated , and then oxycodone  acetaminophen was prescribed . oxycodone  acetaminophen ( oxycodone 5  mg with acetaminophen 325  mg per tablet ) has multimodal analgesia through a combination of an opioid ( oxycodone ) with a nonopioid ( acetaminophen ) component . the intensity and frequency of headache were decreased , and the patient was headache free after about two weeks . immunomodulatory treatments , such as corticosteroids and/or ivig , appear to slow but not halt disease progression , without substantially changing the eventual outcome . during the first year in our case , headaches without any concomitant symptoms could be relieved by rest , like migraines , and did not show any specific characteristics indicating their cause . thus , these headaches could not be identified as prodromal symptoms of rs at that time . only once seizures occurred during the headaches did we consider that rs and the headache were symptoms of the same disease . from then on , headaches and focal seizures were due to the same complaints and , over time , the headache could no longer be relieved without medication . in the final stage , based on the evolution of the headache with the disease progression , we conclude that the initial headache was a presenting symptom of rs . here , we reported an rs case that started with repetitive headaches , adding to the current body of knowledge about rs . rasmussen syndrome ( rs ) without seizures is considered a potentially under - recognized cause of progressive unilateral neurological deficits in childhood . thus , it is important to diagnose atypical cases , even if we can presently only delay but not halt rs progression . this requires recognizing early possible signs and making follow - up observations . in our case , follow - up was inadequate for the first two years because the patient 's parents did not understand the natural history of the disease process . regular follow - up was performed only after confirmation of cerebral lesions identified on brain mri . because of the outcome of rs , in cases involving differential diagnoses of headaches , it is important to consider some rare diseases , such as rs . we think that follow - up for headache patients is important to verify the cause or diagnosis . in our experience , we suggest that repeated brain mri and eeg examinations should be considered to re - evaluate the headache causes even when the examinations were normal previously . in addition to re - evaluating patients with ancillary testing when new symptoms arise such as with new onset seizures . our single case can not serve as an example of what is typical , but our observations suggest that the initial syndrome of rs may involve only headache . though while rs is not presently curable , we hope to help promote early diagnosis and prompt treatment to delay disease progression .
objectivethe aim of this case report was to study that headache maybe the initial symptom in rasmussen 's syndrome ( rs).introductionheadache has not yet been reported as prodromal symptom.methodswe studied a case of rs in which the patient experienced a recurring headache for about one year prior to the onset of partial seizures.resultsmagnetic resonance imaging ( mri ) results were normal when the headache first occurred and showed left brain atrophy three years later . 
 it was difficult to relieve the patient 's headache , even once seizures were controlled.conclusionsthis case demonstrates that the initial symptom of rs may involve only headache .