Saleh Othman

Hemiconvulsion-hemiplegia-epilepsy syndrome A clinical, electroencephalographic and neuroradiological study

Abstract Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1–4 years. These took the form of hemiconvulsion in three of the children and generalized tonic–clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.

Features of hepatic cavernous hemangioma on planar and SPECT Tc-99m-labeled red blood cell scintigraphy

PURPOSE To determine the value and diagnostic accuracy of planar and SPECT Tc-99m-labeled red blood cell (Tc-99m RBC) scintigraphy in the investigation of suspected hepatic cavernous hemangioma as found on ultrasound. PATIENTS AND METHODS One hundred patients, 89 women (89%) and 11 men (11%), between 22 and 67 years old (mean age, 38 years) were evaluated for liver masses found on abdominal ultrasound. All the patients had previous Tc-99m RBC scintigraphy. The size of the lesions ranged from 1 to 9 cm. The final diagnosis was reached through cytologic biopsy, or histopathologic tests (or all of these). RESULTS One hundred thirty lesions were found. With regard to the distribution of the lesions, 116 (89%) were single; 14 (3%) were multiple, with 118 (91%) in the right lobe and 12 (9%) in the left lobe; 83 (64%) were posterior; and 47 (36%) were anterior. With respect to the pattern of radioactivity fill-in, 86 (66%) lesions were typical and 44 (34%) were atypical. The results of Tc-99m RBC scintigraphy showed sensitivity, specificity, and positive and negative predictive and accuracy values of 97%, 83%, 98%, 77%, and 96% respectively. The use of SPECT improved the sensitivity value by 11% for lesions of 1 to 2 cm. CONCLUSIONS Tc-99m RBC scintigraphy is the noninvasive technique most helpful in the diagnosis of hepatic hemangioma, especially in those at risk for lesion rupture or bleeding. SPECT should be performed whenever planar imaging fails to show the lesion by 2 hours. The use of ultrasound should precede scintigraphy for two important reasons: the size and the location of the lesion. Location of the lesion (anterior or posterior) is important for optimal gamma camera acquisition parameters. Lesions less than 1 cm are cannot be detected because they are beyond the limit of spatial resolution of the gamma camera we used.

Renal cortical imaging in children: 99mTc MAG3 versus 99mTc DMSA.

Objective: To determine the potentials of technitium-99m mercaptoacetyltriglycine (99mTc MAG3) to replace technitium-99m dimercaptosuccinic acid (99mTc DMSA) in assessment of renal cortex and split renal function (SRF). Material and Methods: This a retrospective study in which we reviewed the scintigraphic results of 52 patients with age range of 7days–10 years (mean, 5.3 years). A total of 104 studies (1 99mTc MAG3 and 1 99mTc DMSA per patient) were performed between 1 January, 2009 and 31 December, 2010 because of various renal disorders. Both studies were performed within 24 hours of each other, starting with 99mTc MAG3. Results: Twenty patients had normal SRF, and 26 patients had abnormal SRF (6 with solitary kidney, 4 with 1 kidney SRF <10%, and 16 with 1 kidney SRF >10%). In all 46 patients, SRF assessed by 99mTc MAG3 correlated with that by 99mTc DMSA (P = 0.0001). In remaining 6 patients, the correlation was less optimal, and the diseased kidney had overestimation of SRF by 99mTc MAG3. Nonvisualized kidneys and cortical defects were observed equally on both studies. Conclusion: 99mTc MAG3 provides adequate images for assessment of renal cortex and accurate measurement of SRF comparable with 99mTc DMSA results. In addition, it provides important information on the urodynamics of both kidneys, avoids unnecessary radiation to the children, as well as is time saving. We consider it is time to replace the 99mTc DMSA with 99mTc MAG3 in most nephrourologic disorders in pediatric patients and to keep the former to doubtful cases obtained using the latter.

Bilateral tibial adamantinomas simulating stress fractures on scintigraphy.

A 35-year-old woman presented with continued tenderness in the left leg for more than 2 months. The patient was sent to the nuclear medicine department for evaluation. Three phase blood flow imaging followed by a whole body bone scan were performed. Scintigraphy revealed metabolically active lesions in both tibiae. The lesion was larger in size on left side. Scintigraphy per se raised suspicion of bilateral tibial stress fracture. Slightly increased uptake of tracer in the medial condyle of the right femur was because of old trauma. Evaluation of the whole body bone scan showed another lesion in the left pubic bone. Correlation of the CT scan findings of both tibiae (diaphyseal involvement, areas of lucency, breach of cortex, soft tissue component extending into medullary region) with the whole body bone scan raised the suspicion of adamantinoma, which ultimately proved to be multifocal adamantinomas. Differential diagnosis includes fibrous dysplasia, eosinophilic granuloma, polyostotic osteomyelitis, and skeletal metastatic lesions. (Lädermann et al, Orthopedics. 2008;31:282; Daffner et al, Am J Roentgenol. 1982;139:943–948; Desai et al, J Surg Oncol. 2006;93:429–433; Papagelopoulos et al, Orthopedics. 2007;30:211–215; Siblerstein et al, Diagnostic Patterns in Nuclear Medicine. Reston, VA: Society of Nuclear Medicine; 1998:199–210). This is a rare case of multifocal adamantinomas. This case emphasizes that nuclear medicine physicians should not block their minds to a single entity, and always keep options open. Whenever feasible and possible, the whole body should be scanned. Careful review of the whole set of data and correlation with other imaging modalities helps most of the time to reach a proper diagnosis. Adamantinoma should be included in the gamut/differential diagnosis of tibial stress fractures.

Sarcoidosis presenting with massive splenomegaly in a child with a history of iridocyclitis and sensorineural deafness.

Childhood sarcoidosis is a rare multisystemic disorder that can have variable clinical presentations. A triad of skin, eye, and joint involvement is common in children younger than 5 years; however, pulmonary disease is more common in older children, similar to adults. The authors report the case of a 10-year-old girl who presented solely with massive splenomegaly. Her history was significant for iridocyclitis and unilateral sensorineural deafness at 6 and 7 years of age, respectively. A gallium scan showed diffuse splenic uptake, and the pathology of the spleen was consistent with a noncaseating granuloma, with no evidence of malignancy. A work-up for infectious etiology was unremarkable. This case demonstrates that the challenge in diagnosing sarcoidosis in young children stems from its ability to present in several unique clinical scenarios. It also reinforces the importance of tissue evaluation and the exclusion of other differential diagnoses, such as lymphoma, to confirm the diagnosis of sarcoidosis.

Absent right iliac bone on Tc99m MDP bone scan in a patient with Gorham's vanishing bone disease

Gorhams (vanishing bone) disease is an extremely rare condition of the bone. The diagnosis is usually made on the basis of the characteristic history of osteolysis and failure of bone healing in conjunction with the histological findings of marrow fibrosis and increased vascularity. When the disease is established, an X-ray and magnetic resonance imaging show complete loss of affected bone. There are very few reports found in literature on bone scan appearance of the disease. A bone scan of a 24-year-old female patient with known Gorhams disease revealed absence of tracer uptake in the right iliac bone, right sacroiliac joint, and part of the right ischial pubic rami, which matched the radiographic abnormalities. Consequently this disease should be added to the gamut of cold defects seen on bone scan.

A Study on Herpes Simplex Encephalitis in 18 Children, Including 3 Relapses

Background: Herpes Simplex Virus (HSV) is the most common cause of acute sporadic focal encephalitis. Early Diagnosis is, therefore, crucial for predicting outcome. Improved laboratory technology and improved neuroimaging accessibility have enhanced our ability to diagnose this condition. Aims: To assess the reliability of different investigative tools in diagnosing and subsequent management of herpes simplex encephalitis (HSE); as well as the impact of infection and its relapse on the outcome of a cohort of 18 children evaluated during a period of 13 years. Methods: This combined prospective and retrospective study describes the clinical, laboratory, electroencephalographic and diagnostic imaging studies; and outcome in a cohort of 18 children with HSE over a period of 13 years. It also details the clinical and diagnostic features of 3 patients who relapsed. Results: The commonest initial presenting symptoms and signs were fever (100%), seizures (72%) irritability (50%) and weakness/hemiparesis (39%). Cerebrospinal fluid (CSF) pleocytosis was found in 62%, red blood cells (RBCs) >10x10 6 /L in 81% and raised proteins (>0.59g/L) in 52%. Examination for herpes simplex virus (HSV) by polymerase chain reaction (PCR) was positive in 50% (6/12). Electroencephalographic changes were universally abnormal (17/17; 100%) and periodic lateralization discharges (PLEDS) were seen in 35% (6/17). During the acute stage (days 1-8 from symptom onset), magnetic resonance imaging (MRI) revealed abnormalities in 91% (10/11), cranial computed tomography (CT) in 50% (5/10) and single photon emission computed tomography (SPECT), within 3days from onset of symptoms) had significant association with poor outcome (P = 0.002). Initial negative PCR results may become positive on subsequent CSF specimen. Conclusion: Diagnosis of HSE requires combined clinical, laboratory, electroencephalographic and neuroimaging studies. Negative results of PCR do not exclude the infection and should not interrupt the treatment. Early diagnosis and initiation of treatment minimize the devastating effect of HSE. Full course treatment with acyclovir for 21 days is also crucial for prognosis and prevention of subsequent relapse.

Technetium-99m methylene diphosphonate bone scan in the evaluation of bonyache after performing Hajj and/or Umra

Background: During Hajj and Umra the pilgrim will be exposed to tense exercise he/she may not be used to in daily life. This may lead to several musculoskeletal injuries often revealed by clinical and radiological examination however, in some patients further investigations such as bone scan may be required. Aims: The aim of this report is to highlight the potential value of bone scan in the work up of pilgrims with bony ache developed after performing Hajj and/or Umra and in whom routine clinical and radiological examination failed to reveal the cause of their symptoms. Materials and Methods: Twenty three Saudi patients, 18 females with age range 28-70 years (mean = 58 years) and 5 male patients with age range 36-75 years (mean = 49 years) were included in the study. All patients were referred for bone scan within 1-month after performing Hajj and/or Umra. Three phase bone scan is the standard procedure to be performed. The procedure is performed using 700 MBq (20 mCi) technetium-99m methylene diphosphonate and a dual head gamma camera (Bright view from Phillips) equipped with low energy general purpose collimator. Results: Different patterns of bone scan were identified for each type of injury. Seventeen patients (14 females and 3 males) had positive bone scan (74%) and bone scan was negative in 4 female and 2 male patients (26%). Conclusion: The percentage yield of bone scan in evaluating the Muslim pilgrim with bony ache after performing Hajj and/or Umra is quiet high and thus bone scan can be recommended in the work up of those patients in whom clinical and radiologic examination failed to reveal the cause of their symptoms. Dual energy X-ray absorptiometry scan is also advised in patients with suspected insufficiency and stress fractures.

Acute lobar nephronia in renal transplant: Gallium-67 scintigraphy for diagnosis and therapy monitoring.

A 33 years old female patient with chronic renal transplant rejection proved by MAG3, ultrasound and graft biopsy presented with abdominal pain and fever. Part of her work up included gallium-67 scan which revealed diffuse abnormal graft uptake with multifocal areas of marked uptake. Findings were interpreted as acute lobar nephronia. Repeat gallium scan two weeks after intravenous antibiotic therapy showed significant response reflected by resolution of most of focal areas of increased uptake which was parallel to clinical improvement.

Right upper quadrant pain with normal hepatobiliary ultrasound: Can hepatobiliary scintigraphy define the cause?

Background/Aim: The objective of this study was to assess the value of hepatobiliary scintigraphy (HS) for the diagnosis of right upper quadrant (RUQ) abdominal pain in patients with normal hepatobiliary ultrasound (HU). This is an observational study with a retrospective analysis of data from March 2008 to August 2010. Materials and Methods: We reviewed the HS results of 30 patients, aged 29–69 years (average 45.8 years); 12 male and 18 female patients. Patient selection to perform the HS was RUQ abdominal pain, suspected hepatobiliary disorder, and negative HU. All patients had gone through the standard procedure of HS. Results: Based on predefined interpretation criteria, HS results were divided into 2 patterns: Normal (n=8, 25.8%) and abnormal (n=22, 73%): 18 patients (81.8%) having early gallbladder (GB) and common bile duct visualization, and delayed transit to small bowel (SB), which can be seen only after a fatty meal with normal or abnormal GB ejection fraction (GBEF) pattern characteristic of Oddis sphincter dyskinesia. The remaining 4 patients (8.18%) had acalculous cholycystitis pattern: Delayed GB visualization with activity appearing in SB before GB. Conclusion: HS with fatty meal stimulation and GBEF estimation seems to be a reliable test, which may reveal a biliary cause in more than 70% of patients with RUQ abdominal pain and normal HU. Normal results exclude functional biliary cause. The decision for invasive or noninvasive therapeutic approach may depend on the results of HS.