Clinical History
An 8-year-old male patient with 49, XXXXY syndrome was referred to the radiology department as he had two focal seizures in the last 3-days and a 1-week history of headache in left temporal region.
Imaging Findings
An 8-year-old male patient with 49, XXXXY syndrome was seizure-free since he was given a diagnosis of epilepsy and was put on an antiepileptic therapy at age 5. He was referred to the radiology
department as he had two focal seizures in the last 3-days and a 1-week history of headache in left temporal region. Fever for one week under antibiotic therapy, weakness were achieved in the
history. No significant change was observed in his mental status. Physical examination revealed hypertrophy of the tonsils, hyperemia of the oropharynx, nuchal rigidity and mucocutaneous eruptions at
the tip of the nose. Cranial CT and MR imaging with a 3T MR scanner was performed.
Discussion
Cranial CT examination showed acute hemorrhage at left temporal lobe. Associated cortical thickening with hypodensity and sulcal effacement suggestive of edema or ischemia at left temporal lobe were
observed (Fig 1). MR imaging showed acute-early subacute hemorrhage in the left medial temporal lobe, left Sylvian fissure and in both lateral ventricles. Cortical swelling and hyperintensity on
T2-WI as well as profound meningeal and cortical enhancement predominantly on the left temporal lobe on postcontrast T1-WI was present. Meningeal enhancement was diffuse over the cerebral hemispheres
and cortical involvement was extensive in the left cerebral hemisphere including posterior parietal and occipital lobes (Fig 2A, B). DWI revealed mild increased cortical intensity that is
attributable to T2-shine through effect. Calculated ADC values were 1.15 x 10-3, 1.03 x 10-3, 0.86 x 10-3 mm2/sn in the left temporal lobe from anterior to
posterior and were 0.96 x 10-3, 0.90 x 10-3, 0.86 x 10-3 mm2/sn correspondingly in the contralateral normal appearing parenchyma. Accordingly, anteromedial
temporal lobe showed restricted diffusion (0.54 x 10-3 mm2/sn, contralateral 0.91 x 10-3 mm2/sn) resulting from blood both in the parenchyma and
subarachnoid space, while other affected areas had increased diffusion consistent with vasogenic edema (Fig 3A, B). Decreased caliber of the left distal ICA shown by MR imaging (Fig 4). An arachnoid
cyst in the interpedincular cistern was observed in the patient incidentally (Fig 2A).
Hemorrhagic temporal lobe lesions in the appropriate clinical setting is highly suggestive of HSE; however, although consistently present at pathological specimens, hemorrhage is infrequently seen by
imaging, especially CT (1). Analysis of CSF obtained from a non-traumatic tap reveals excessive red blood cells (>500/mm3) besides pleocytosis in approximately 20% of patients with
acute encephalitis. Typically associated with necrotizing and hemorrhagic encephalitis (2). Apart from necrotizing nature of the disease, possibly accompanying vasculitis as with varicella zoster
might have caused SAH. Manifestation of circumferentially symmetrical narrowing of the left distal ICA by MR studies could give a support for infectious vasculitis in this case (3). It is
consistently shown that MR imaging is more sensitive than CT for detection of cerebral lesions of HSE (2, 4). HSE in children and adults could be hemorrhagic with initial findings restricted to the
medial and inferior temporal lobes. Imaging often displays unilateral findings, with a spread to contralateral side not infrequently. Involved areas appear hypointense on T1-WI and hyperintense on
T2-WI on MR imaging unless hemorrhage accompanies (2). Recently, DWI proved to be superior to ‘conventional’ images, providing more information on affected brain tissue, and especially in
cases with restricted water molecular motion. Showing the type of the edema (cytotoxic vs. vasogenic) in HSE by calculation of ADC values has been found important as it might have a prognostic value
on the clinical outcome (4, 5). In a study by Sener RN, the cases which DWI showed predominantly cytotoxic edema were in severe clinical condition, while others had a better outcome following prompt
therapy (4).
Differential Diagnosis List
Herpes Simplex encephalitis
Final Diagnosis
Herpes Simplex encephalitis