acute hemorrhagic leukoencephalitis ( ahle ) is a rare fatal disease presenting with an acute onset of neurological abnormalities .
it is categorized in a group of diseases called acute disseminated encephalomyelitis ( adem ) . in this disease ,
an acute rapidly progressive hemorrhage in the white matter leads to a fulminant clinical course .
the first presentations are fever and a rapid deterioration of consciousness , but it may finally lead to death within a few days . the acute onset of a rapidly progressive neurological disorder with asymmetric involvement of the brain , polymorphonuclear predominant peripheral leukocytosis , cerebrospinal fluid ( csf ) pleocytosis , and specific findings in the serial computed tomography scans or magnetic resonance imaging ( mri ) are the main criteria for the diagnosis .
the areas involved in the majority of the cases are the parietal lobes , but the lesions can be seen in the subcortical white matter , mid brain , pons , corpus callosum , basal ganglia , medulla , cerebellum , and even spinal cord .
the imaging techniques may also complicate the diagnosis as computed tomography and mri might show nonspecific findings such as hematoma in the basal ganglia and ventricles .
in addition , symmetrical low density areas in the bilateral white matter might result in a false preliminary diagnosis of hemorrhagic cerebrovascular disease . in this case report , we present a case of ahle in children .
a 13-year - old girl with loss of consciousness and right - sided parotiditis was referred to our center , imam reza hospital , mashhad university of medical sciences , from a hospital in herat ( northern afghanistan ) .
she had been admitted to that center 4 days previously because of fever and loss of consciousness . according to the girl s mother ,
the right side of her face was swollen on the evening of the first admission .
the loss of consciousness had begun with drowsiness , which had progressed to unresponsiveness to the verbal stimuli and poor response to painful stimuli by midnight .
eventually , the patient had become completely unresponsive to any stimuli in the hospital in herat . after the initiation of supportive measures and performance of lumbar puncture , intravenous ceftriaxone , vancomycin , and acyclovir ( 30 mg / kg / d ) was commenced for the patient .
after she was admitted to our center , an informed consent was obtained from her parents .
her consciousness deteriorated even more , and she became completely unresponsive to verbal stimuli . within 2 days , she developed respiratory failure and a decerebrated posture and subsequently spasticity of the four limbs and rigidity of the left leg .
her initial mri revealed multifocal hemorrhages without edema in the right temporal white matter ( figure 1 ) . in complete blood count
lumbar puncture performed in the hospital in afghanistan , the csf showed an elevated white blood cell count ( 3 - 4 cells / mm ) and an increased protein level ( 69 mg / dl ) .
the culture of the csf regarding bacterial and viral infections was negative , although there were some limitations to serological tests , including deficit of technical instrumentations in our hospital .
the result of immunological and microbiological studies , comprising hepatitis b surface antigen , anti - hepatitis a virus and hepatitis b virus , and polymerase chain reaction for herpes simplex virus , were all negative .
the laboratory tests for mycoplasma and tuberculosis as well as the csf culture were also negative .
coagulation tests such as bleeding time , prothrombin time , partial thromboplastin time , and platelet count were normal .
no abnormality was found in electrolyte analysis and biochemical studies such as blood sugar and liver function tests .
a second mri ( 20 days later ) showed multiple hemorrhagic lesions in the pons and thalamus of both sides ( figure 2 ) .
our diagnosis was according to clinical , radiological , and laboratory findings , and there were no pathological examinations to confirm it .
there are multiple hemorrhagic lesions in the pons and thalamus of both sides . with a diagnosis of ahle ,
our patient was treated with methylprednisolone ( 20 mg / kg pulse therapy ) , intravenous immunoglobulin ( ivig ) , acyclovir ( 30 mg / kg / d ) , and plasmapheresis ( 3-hour sessions for 3 times ) .
after 29 days stay in the pediatric intensive care unit and under supervision of pediatric toxicologists and neurologists , she was extubated and attained a remarkable healing .
her mri demonstrated improvement ( figure 3 ) . at one year s follow - up , the treatment was completely successful and neurological examinations , comprised of mental status , motor skills , sensory skills , balance and coordination , reflexes , and functioning of the nerves , were all normal .
ahle is a rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter .
it is a more severe form of adem and is characterized by a fulminant clinical course .
it is usually preceded by an infectious illness such as measles , mumps , rubella , and respiratory infections .
nevertheless , no virus or other infectious agent has been isolated from the csf or brain in case of adem .
considering clinical and imaging findings , the differential diagnosis of this disease are hemorrhagic cerebrovascular disease , infectious encephalitis , meningitis , vasculitis , fulminant multiple sclerosis , other causes of adem , and venous sinus thrombosis .
most of the patients experience a viral , bacterial , or parasitical infection and usually have fever at the time of admission . however , infectious encephalitis and meningitis are similar to ahle as they are both usually preceded by an infectious disease such as measles , mumps , and respiratory tract infections .
our patient had parotiditis on admission , which is in favor of a viral infection .
in two different reports , befort and francisci presented cases of ahle after epstein - barr virus infection in adults . nonetheless , as can be observed in our patient ,
besides differences in the clinical presentations and course of the disease , vasculitis usually manifests on mri as smaller multiple lesions that are both in the cortex and in the white matter .
moreover , serological tests for collagen diseases , which are used for the diagnosis of vasculitides , were negative in our patient .
fever , leukocytosis , and csf features , which were seen in our patient , are not compatible with the diagnosis of acute fulminant multiple sclerosis .
the absence of oligoclonal bands is another characteristic for the exclusion of acute fulminant multiple sclerosis .
it is very difficult to distinguish other causes of adem from ahle based on clinical and mri findings , and diagnosis is basically made via biopsy .
however , there are certain epidemiological , clinical , laboratory , and pathological distinctions between them . for instance
, adem is mainly a disease of children , whereas ahle is more common in young adults .
in addition , ahle presents more acutely and has larger and more edematous lesions with features of hemorrhage . in our patient ,
several findings , including acute fulminant course of the disease , a history of parotiditis , csf profiles , indicative immunological and bacteriological tests , and mri results , strongly supported the diagnosis of ahle .
previous studies have shown that treatment with iv methylprednisolone , ivig , acyclovir , and plasmapheresis are helpful and can be life - saving . following the same protocol , our patient recovered and was extubated .
still , as there is evidence that ahle might have a biphasic presentation , our patient should be followed up for a longer time period with several mris .
ahle is a rare and severe demyelinating disease , the mortality and morbidity of which can be lessened by early detection and treatment with steroid therapy , ivig , acyclovir , and plasmapheresis .
 acute hemorrhagic leukoencephalitis ( ahle ) is a rare demyelinating disease characterized by an acute rapidly progressive fulminant inflammation of the white matter . in this case report 
 , we introduce a case of ahle in children with an interesting and lengthy process and successful treatment.a previously healthy 13-year - old girl was admitted to the hospital because of fever and loss of consciousness . 
 after 4 days , she was referred to our pediatric intensive care unit in mashhad , iran . on admission , she had right - sided parotiditis . with a diagnosis of ahle , 
 our patient was treated with methylprednisolone , intravenous immunoglobulin , acyclovir , and plasmapheresis.ahle is a rare and severe demyelinating disease , the mortality and morbidity of which can be decreased by early detection and treatment with steroid therapy , intravenous immunoglobulin , acyclovir , and plasmapheresis . 
Introduction
Case Presentation
Discussion
Conclusion