MRI and MR spectroscopy findings of a case of subacute sclerosing panencephalitis affecting the corpus callosum.

Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressive, fatal, inflammatory and neurodegenerative disease that is seen mostly in children and young adolescents, and primarily affects the parieto-occipital lobes. The corpus callosum, cerebellum and basal ganglia are less frequently involved. MR spectroscopy (MRS) may illustrate the pathophysiological features of SSPE. To the best of our knowledge, this is the second report of MRS findings of corpus callosum involvement in a stage 3 SSPE case.

Cerebral atrophy and subdural haemorrhage after cerebellar and cerebral infarcts in an 8-month-old child after having been stung by a scorpion.

A scorpion sting causing cerebellar and cerebral infarctions with corpus callosum involvement and bilateral cerebral atrophy with subdural haemorrhage in an 8-month-old girl, has not been previously described to the best of our knowledge.

[A case of brucellosis misdiagnosed as Crimean-Congo hemorrhagic fever]. / Kirim-Kongo Kanamali Atesi ile Karisan Bir Bruselloz Olgusu.

Brucellosis which is a zoonotic infection, is an important public health problem in Turkey and all over the world. The disease may involve many organs and systems. Since the symptoms of brucellosis are non-specific, difficulties in differential diagnosis and misdiagnosis are frequent. In this case report we present a case of brucellosis, misdiagnosed as Crimean-Congo hemorrhagic fever (CCHF). A 13-year-old boy was referred from another medical center with preliminary diagnosis of CCHF and admitted to our clinic with fever and a history of presence of a tick on his back. His physical observation only included splenomegaly. The laboratory results on admission were anemia, thrombocytopenia, elevation of acute phase reactants and liver transaminase levels. Abdominal ultrasonography revealed splenomegaly. Since the patient had anemia, epistaxis, fever and thrombocytopenia, he was initially diagnosed as CCHF. Meantime serum sample of the patient had been sent to Refik Saydam National Public Health Agency for CCHF PCR test. The fever of the patient could not be controlled. His detailed medical history revealed stockbreeding and consumption of raw milk products. Patient's signs and symptoms were also compatible with brucellosis and standard tube agglutination test for brucellosis was positive at 1/1280 titer in serum. The patient was diagnosed as brucellosis and the treatment was started with combination of rifampicin (1 x 600 mg/day) and doxycycline (2 x 100 mg/day). Blood cultures yielded negative result. The PCR tests for CCHF was found also negative. His fever and other complaints improved with treatment which was completed in six weeks and the follow-up was without complications. Turkey is endemic both for brucellosis and CCHF. This case was reported to emphasize that the cases of brucellosis could mimic other diseases and brucellosis should also be considered in the differential diagnosis of CCHF.

Ultrasonographic screening of newborns for congenital anomalies of the kidney and the urinary tracts.

PURPOSE: To search for the efficiency of scanning the newborns with routine urinary system ultrasonography. MATERIALS AND METHODS: Urinary ultrasonography has been carried out on 721 infants born in or brought to our hospital. During the study, name, sex, week of birth, presence of antenatal diagnosis or urinary tract infections, and pathologies in examinations of the babies were recorded. Ultrasonography analysis was done with a scanner by a radiologist. Patients identified to have pathology, were watched closely in pediatric nephrology clinic, and advanced visualizations and treatments were carried out. RESULTS: Seventy-six infants (10.5%) had congenital anomalies of the kidney and the urinary tracts that prompted medical and/or surgical intervention. Of whom, 32 were diagnosed with antenatal ultrasonography and 44 during their initial postnatal ultrasonography screening. The most frequent identified pathology was hydronephrosis, in particular physiologic hydronephrosis (35.8%). The most frequent congenital urinary anomaly which caused hydronephrosis was ureteropelvic obstruction. CONCLUSION: It is suggested to apply the urinary ultrasonography scanning to all the infants that are born or brought to the university hospital. If possible, it is to be considered to include urinary ultrasonography scanning in newborn scanning programs.

Possible pathogenic link between migraine and urotensin-II.

Our aim was to determine the levels of human urotensin-II (hU-II) in the plasma of migraine patients and controls, to ascertain if there were a difference in the pathogenesis of migraine. A total of 27 patients who suffer from migraines and 27 controls were included in the study. Venous blood samples were drawn twice both from migraine patients and controls to measure hU-II plasma levels. The average levels of hU-II during migraine episode, between episodes, and controls were found to be 0.483, 0.493, and 0.737 pg/mL, respectively. The levels of hU-II in the controls were higher significantly. When comparisons were made according to sex, age groups, and types and durations of migraine, there was no significant difference in the levels of hU-II in the patients. The low levels of hU-II in the plasma of migraine patients compared with controls may be an indicator of its role in the pathogenesis.

Isolated preauricular pits and tags: is it necessary to investigate renal abnormalities and hearing impairment?

We investigated the incidence and co-existence of hearing impairment and renal abnormalities in healthy children with preauricular tags and pits. Study population consists of 13,740 primary school children from routine health check. Thirty-five children with preauricular tags and pits were noted. Control group consisted of 91 patients without pits and tags, who underwent renal ultrasound and were scheduled to pediatric outpatient clinic. Urinalysis, renal ultrasound, otoacoustic emission were performed in both the groups. The prevalence of renal abnormality (1/36; 2.7%) and hearing impairment (1/36; 2.7%) in patients with preauricular tags and pits was similar to that of control group (3/91; 3.2% and 4/91; 4.3%) (P = 0.87, P = 0.64, respectively). According to our results, it is not necessary to investigate hearing or urinary abnormality in patients with preauricular tag or pit, unless there is an association of a syndrome or family history of hearing or renal impairment.

Osmotic demyelination syndrome in a 40-day-old infant.

Osmotic demyelination syndrome refers to the myelin destruction of various brain structures that follows osmotic stress. It affects myelinated brain; therefore, it is very rare in babies and it has not been reported in patients younger than 10 months of age. Herein, we present a 40-day-old infant with osmotic demyelination syndrome, along with imaging findings. Her pontine and thalamic lesions regressed during the 10-day treatment course, whereas demyelinated areas in the lentiform nucleus persisted. Magnetic resonance spectroscopy of the latter revealed decreased levels of all major metabolites. Imaging findings remained unchanged after that phase.

Merosin-negative congenital muscular dystrophy: diffusion-weighted imaging findings of brain.

Merosin-negative congenital muscular dystrophy is a rare genetic disease of childhood involving the central and peripheral nervous system. There were high signal intensities throughout the centrum semiovale, periventricular, and sub-cortical white matters on T2-weighted images in a 4-year-old girl with merosin-negative congenital muscular dystrophy. An apparent diffusion coefficient map revealed increased signal intensity and apparent diffusion coefficient values in the periventricular and deep white matters. It may be attributable to increased water content in the white matter because of an abnormal blood-brain barrier rather than to decreased or abnormal myelination.

Can the requirement of a diversion procedure be predicted after an uncomplicated intracranial hydatid cyst surgery?

Intracranial hydatidosis is mainly a pediatric disease. Surgical evacuation of the mass in toto is the gold standard of therapy. No concise data related to the requirement of diversion procedures after uncomplicated hydatid cyst removal is encountered in the literature. The aim of this report is to focus on this idea and estimate the necessity of a diversion procedure beforehand. A 6-year-old girl both with intracranial and renal involvement of hydatidosis underwent surgery. Intact removal of the intracranial cyst harboring daughter vesicles was achieved. A persistent porencephalic cyst at the operative site required a shunt 6 months after surgery. Thick pericyst formation was blamed for this specific progress and the pathophysiology is discussed.

Magnetic resonance spectroscopic characteristics of glutaric aciduria type II.

Magnetic resonance spectroscopy (MRS) of a 12-year-old female patient with glutaric aciduria type II was compared with data obtained from four healthy age- and sex-matched volunteers. In the clinically active phase, conventional magnetic resonance imaging showed mild ventricular dilatation. Frontal lobe choline/creatine (Cho/Cr) ratio (1.98) was higher than the ratios reported for the comparison participants (1.64 [SD 0.21]). The N-acetylaspartate/creatine (NAA/Cr) ratio (1.95) was lower than normal limits (2.66 [SD 0.23]). After successful riboflavin treatment and dietary restriction for proteins, the NAA/Cr ratio was within the normal range (2.44) and Cho/Cr ratio was below the normal range (1.15), suggesting riboflavin-responsive multiple acyl-coA dehydrogenase deficiency. An elevated Cho/Cr ratio and decreased NAA/Cr ratio is consistent with a demyelinating process in the active phase of glutaric aciduria type II. MRS helps to monitor the progress of the disease and the efficacy of treatment by revealing changes in NAA/Cr and Cho/Cr ratios.

Unilateral reverse ocular bobbing caused by tuberous sclerosis.

A female aged 12 months with developmental delay and left-sided reverse ocular bobbing (rapid deviation of the eye upward and a slow return to the horizontal position) was observed. At birth, an upper left gingival mass, pathologically diagnosed as a benign granular cell tumour, was removed. On computed tomography (CT), left middle cerebral pedincular and midpontine lesions were seen. Magnetic resonance imaging revealed additional white matter hamartomas, corticosubcortical tubers, and subependymal nodules. The patient was re-examined at 36 months. Her general developmental quotient was equivalent to 23 months of age. Third cranial nerve functions and auditory brainstem response were normal. Her abnormal eye movements were still present. Reverse bobbing is usually observed in patients who are unconscious and who have significant pontine pathology and disruption of the reticular formation. This case is the first tuberous sclerosis-related ocular bobbing case to our knowledge and is interesting as the causative lesion was relatively mild, and ocular horizontal movements were preserved.

Giant axonal neuropathy: diffusion-weighted imaging features of the brain.

Giant axonal neuropathy is a rare autosomal recessive childhood disorder characterized by a peripheral neuropathy and features of central nervous system involvement. Magnetic resonance imaging (MRI) of an 11-year-old boy with giant axonal neuropathy revealed high signal intensity in the white matter of the cerebrum and cerebellum on T(2)-weighted imaging. An apparent diffusion coefficient map revealed increased apparent diffusion coefficient values in the periventricular, deep, and cerebellar white matter, basal ganglia, and thalamus. Increased apparent diffusion coefficient values in distinct locations suggest increased mobility of water molecules in the brain of a patient with giant axonal neuropathy. This finding could indicate a myelin disorder such as demyelination. Diffusion-weighted imaging should be performed to reveal apparent diffusion coefficient changes and determine brain involvement in patients with giant axonal neuropathy.

Clinical outcomes of neonatal hypoxic ischemic encephalopathy evaluated with diffusion-weighted magnetic resonance imaging.

PURPOSE: Detection of early phase neurological deficit in babies with hypoxic-ischemic encephalopathy (HIE) is the most important step to determine the appropriate preventive treatment methods. Diffusion-weighted imaging (DWI) is the most sensitive radiological modality to detect ischemic changes in the brain, in their earliest phase. Herein, we present the results of our study about the role of DWI in the diagnosis and determining the prognosis of HIE in neonates. MATERIALS AND METHODS: The study included 36 cases (4 preterm, 32 term babies) who were diagnosed with HIE within 24 hours of birth and classified according to modified Sarnat staging. They were examined for the presence of neurological sequelae at 3 and 6 months of age with electroencephalography (EEG), visual evoked potential (VEP), brainstem auditory evoked potential (BAEP), and Denver II developmental screening tests. All 36 patients underwent conventional magnetic resonance (MR) imaging and DWI within the first 24 hours of birth; survivors underwent repetitive imaging exams at the end of the first week and then after a month. RESULTS: Seventeen stage I cases (47%), 12 stage II cases (33%), and 7 stage III cases (20%) were detected. DWI obtained within the first 24 hours showed high sensitivity (100%) in detecting the permanent neurological sequelae but with very low specificity (20%). The negative predictive value of DWI in this period was 100%; however, in DWI obtained at the end of the first month, not only its sensitivity was preserved, but its specificity reached 80%. The negative predictive value of DWI in this period was preserved and the positive predictive value improved. The importance of DWI in detecting sequelae at the end of the first month was also demonstrated by McNemar (p=0.250) and Kappa (Kappa=0.719) tests. There was no difference between conventional MR imaging and DWI in detecting sequelae at the end of first month. CONCLUSION: DWI is superior to other imaging modalities in detecting ischemia; not only because of its high sensitivity in the early phase, but also because of its high sensitivity and specificity in the late phase. Moreover, with its high negative predictive value, DWI can be used for excluding the possibility of sequelae development in the early phase of HIE cases for medico-legal purposes.

Protein C, protein S and other pro- and anticoagulant activities among epileptic children using sodium valproate.

This study was conducted to evaluate some anticoagulants' (protein C, protein S and antithrombin) activities among epileptic children treated with VPA and to learn if thromboembolic events occur among these children or their parents. Twenty-five boys and 15 girls using VPA for at least 3 months were included in the VPA group. The control group consisted of 28 boys and 12 girls who had no infection and did not use any medication that could alter protein C activity. Complete blood counts, ALT, AST, PT, aPTT, fibrinogen, protein C, protein S and AT tests were studied in both groups. Serum VPA levels were determined in the VPA group. Protein C and protein S activities of the children in the VPA group were significantly lower than those in the control group (89.5+/-19.3% vs 104.9+/-21.7% and 44.6+/-16.3% vs 59.4+/-28.4%, respectively). Neither children using VPA, nor their parents had any thromboembolic events in medical history. Platelet counts, ALT and fibrinogen levels in the VPA group were significantly lower than those in the controls. A negative correlation was found between serum VPA level and platelet counts. There were also negative correlations between fibrinogen and serum VPA levels, and between fibrinogen level and protein S activity. The children in the VPA group had lower PT and higher aPTT levels than the children in the control group. Since other factors known to alter the anticoagulant activities and liver functions were eliminated initially, the decreases of protein C and protein S activities, thrombocyte counts, ALT, PT and fibrinogen levels and increase in aPTT level may be attributed to VPA. VPA hepatotoxicity can be the cause of decreased pro- and anticoagulant activities.

Diffusion weighted MR findings of brain involvement in tuberous sclerosis.

PURPOSE: Diffusion Weighted Imaging (DWI) is effective in identifying microstructural cerebral parenchymal changes. We studied the diffusion characteristics of hamartomas and normal white matter in cases with tuberous sclerosis (TS). MATERIALS AND METHODS: Diffusion weighted images of 6 TS cases (ages between 3 - 15 years, mean 9.0 years, SD 4.4 years) that presented to our center for magnetic resonance (MR) imaging have been retrospectively evaluated. In addition to 56 non-calcified hamartomas of TS patients, apparent diffusion coefficient (ADC) values measured from frontal, parietal normal white matter, and basal ganglions of TS patients were compared with values obtained from 9 normal subjects (ages 1 - 13 years, mean 8.9 years, SD 3.4 years). Hamartomas were divided into 3 subgroups based on their locations, and the ADC values measured in these groups were compared. RESULTS: ADC values of all hamartomas were between 838 and 2230, with a mean value of 1408 mm (2)/sec x 10(-3) (SD: 273.2); ADC values of the white matter of normal subjects were between 695 and 857, with a mean value of 776.1 mm (2)/sec x 10(-3) (SD: 44.23) (p < 0.0001). ADC values of subependymal nodules, white matter hamartomas, and subcortical tubers were 838-2230 (mean: 1440.5 mm (2)/sec x 10(-3); SD: 526.46), 1046-1622 (mean: 1328.6 mm (2)/sec x 10(-3); SD: 189.4), and 981-1973 (mean: 1417.4 mm (2)/sec x 10(-3); SD: 219.5), respectively (p = 0.666). CONCLUSION: Diffusion characteristics of white matter hamartomas resulting from TS clearly differ from those of normal white matter, but no significant difference was observed in ADC values of these lesions based on their locations. Moreover, the ADC measurements of normal white matter in these cases did not differ from those of the control group, indicating that the disease does not cause a common explicit damage in white matter and central gray matter, other than hamartomas, which can be detected by DWI. DWI may only be used in the differential diagnosis of hamartomas from secondary lesions with T1 and T2W signal intensities similar to those of hamartomas and with different diffusion characteristics.

Auditory brainstem response in pediatric migraine: during the attack and asymptomatic period.

OBJECTIVE: The aim of this study was to evaluate the hearing parameters of children with migraine during ictal and interictal period. METHOD: 16 pediatric patients with migraine and normal otolaryngologic examination were evaluated. Hearing parameters were assessed with auditory brainstem response (ABR) testing between and during the migraine attacks. Binaural absolute latencies of waves I, III and V, interpeak latencies I-III, III-V and I-V of ABR in response to 80 dB nHL clicks were calculated. Initial findings were compared with those of 20 healthy volunteers. RESULTS: Peak latencies of wave V and interpeak latencies of I-V were prolonged during the attack in migraineurs on the left. The side of latency elongation was not affected by the side of headache. When these parameters were separately compared for gender, they were prolonged in boys during the attack in migraineurs; however in girls, while there was statistically significant difference at interpeak latencies of I-V, no significant difference was noted at peak latencies of wave V. CONCLUSIONS: ABR waves did not exceed clinical norms in migraine patients in headache-free period. But, important effects on sensorineural hearing parameters were detected during the attack. Our results indicated a transient impairment of the auditory brainstem function during the headache in pediatric migraine patients.

Subacute sclerosing panencephalitis: relationship between clinical stage and diffusion-weighted imaging findings.

PURPOSE: To investigate the relationship between clinical stages and apparent diffusion coefficient (ADC) changes in the brain of patients with subacute sclerosing panencephalitis (SSPE). MATERIALS AND METHODS: A total of 18 patients with stage II (N = 11) and III (N = 7) SSPE and 11 age-matched controls underwent routine MRI and diffusion-weighted imaging (DWI). The ADC values were automatically calculated. Seven distinct neuroanatomic structures (frontal, parieto-occipital, and cerebellar white matter; deep white matter; thalamus; basal ganglia; and brainstem) were selected for analysis in the patient and control groups. RESULTS: Hyperintensities in the periventricular and subcortical white matters on T2-weighted images and ADC maps were detected in 63.6% of patients with stage II and in all patients with stage III. There were significant differences between stage II and III patients and also between patients and control group in ADC values that obtained from all locations. The highest mean ADC values were calculated in stage III patients. Although MRI and DWI findings were normal in four patients with stage II disease, ADC values were significantly increased when compared with controls. CONCLUSION: The stage of disorder may be independent of DWI appearance during the early stage (stages I and II), even though the brain is affected. Therefore, DWI and ADC values supplemental to routine MRI should also be utilized for lesion detection and definition to enhance diagnostic accuracy in patients with SSPE.