Hungarian Radiology

[Early detection of adult femoral head necrosis]

GION Katalin, PALKÓ András

APRIL 10, 2005

Hungarian Radiology - 2005;79(02)

[Adult avascular femoral head necrosis is common in young adulthood, and in 80% of cases affects male patients. The disease is bilateral in 40-80 %, and it may take several years to develop on the contralateral side. Late diagnosis and lack of early therapy can cause progressive disease and finally movement restraint. The diagnosis in early stage is crucial for choosing the most effective strategy in therapy. It is important to be aware of pathogenesis, clinical course and the differential diagnostic options of the disease, and these should be associated to the diagnostic findings at different imaging modalities. Based on this concept, we conclude that MR examinaton is the method of choice for the early (reversible) stage assessment. MRI of the hip is also able to evaluate and follow up the healthy contralateral side without further strain.]

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[A small piece of renaissance]

LOMBAY Béla

Hungarian Radiology

[Radiological diagnostics of the pancreas neoplasms - Onco Update 2005]

FORRAI Gábor, BODOKY György

[Authors reviewed the recent results of pancreas tumour radiological diagnostics and the place of the imaging and interventional methods. Systematical review of the most recent articles were summarized (July 2003-December 2004) in the following subjects: the etiology and clinico-pathology, general diagnostic and therapeutical questions of early pancreatic neoplasms, abdominal ultrasound, computed tomography, multidetector computed tomography, magnetic resonance imaging, MR-cholangiopancreatography, endoscopic retrograde cholangiopancreatography, endoscopic ultrasound, intraductal ultrasound, endoscopic ultrasound-guided cytology, percutaneous biopsy, positron emission tomography, positron emission tomography - computed tomography, special pancreatic tumours. Experiences about the pancreas diagnostic methods are accumulating year-to-year rapidly. Therefore the current examination algorithm is changing continuously. New diagnostic and therapeutic modalities are entering in the daily practice. These are the reasons why the up-to-date knowledge of the literature is mandatory.]

Hungarian Radiology

[Radiologic diagnosis of the diseases of the pediatric gynecology]

LÓRÁND Ágnes, HARKÁNYI Zoltán, LOVAS Györgyi, HÉJJ Ildikó

[The basic examination of the pediatric pelvic organs is the transabdominal ultrasound which provides useful information about the anatomy and the pathological changes and in the vast majority of cases it is sufficient for treatment planning and to establish the diagnosis. Additional examinations are needed in case of complex developmental anomalies, in suspition of tumor, in staging and follow up examinations of tumors. Among the modern imaging methods the use of CT and MRI can be considered. The authors described the most frequent diseases in their practice and gave a brief overview on anatomical and physiological basics which is necessary for the exact interpretation of the examinations.]

Hungarian Radiology

[Dudoenum obstruction caused by duodenal diaphragm]

RUDAS Gábor, SEKYRA Pavel, PUMBERGER Wolfgang, POVYSIL Brigitta

[INTRODUCTION - Duodenum obstruction is a rare gastrointestinal developmental anomaly. It may be complete or incomplete. The incomplete or intrinsic form is often caused by an intraluminal membrane or web duodenal stenosis. The passage is existed with the help of a small perforated lake. Clinically the leading sign is the vomiting. CASE REPORT - A three days old baby admitted because of vomiting. Abdominal X-Ray and US could not found any reason of vomiting but the gastrointestinal contrast series had diagnostic value. Surgery proved the radiological diagnosis. CONCLUSION - For the diagnosis the conventional XRay and ultrasound is not sufficient in every case, the gold standard is the gastrointestinal contrast examination.]

Hungarian Radiology

[Czech dysplasia metatarsal type]

IVO Marik, MARIKOVA Olga, DANA Zemkova, MIROSLAV Kuklik, KAZIMIERZ Kozlowski

[We report a further female patient with the recently described new bone disease, Czech dysplasia metatarsal type. Czech dysplasia metatarsal type (CDMT) is an autosomal dominant debilitating disorder. Its constant phenotypic trait is hypoplasia/ dysplasia of the 3rd and/or 4th toes. “Congenital hip dysplasia” or “hip disease” is commonly evoked in the family history. The clinical course may be severe, incapacitating the patients early in life, or progress slowly with increasing hip and spine pain. This girl's phenotype and radiographic findings are similar to the seven previously reported cases.]

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[Fournier’s gangrene]

MÁTÉKA Ilona, MORVAY Zita, PALKÓ András

[INTRODUCTION - Fournier’s gangrene is a rare, rapidly progressive specific form of necrotizing fasciitis that involves the soft tissues of the genital and perianal regions. The infection may spread from these regions along the abdominal wall or towards the lower limbs and has a very high mortality rate. History of the patient, the clinical features and the imaging techniques all together help to recognize the condition and to localize the affected area. CASE REPORT - We present the case of a thirty-year-old male patient, who had on admission a swollen left lower limb. Deep venous thrombosis was excluded by Doppler us. A few days later an other us examination revealed the fasciitis of the left thigh and the scrotum, originating from the deep gluteal bed-sore of the patient. A CT was performed to define the extent of the process and help the planning of the surgical intervention. The patient has recovered well after the operation. CONCLUSION - Due to the high mortality rate of Fournier’s gangrene it is essential to recognize this condition in time and to provide the appropriate surgical intervention or antibiotic therapy. Imaging modalities play a very important role in the diagnosis of Fournier’s gangrene.]

Clinical Neuroscience

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

Clinical Neuroscience

Cholinesterase inhibitors and memantine for the treatment of Alzheimer and non-Alzheimer dementias

BALÁZS Nóra , BERECZKI Dániel, KOVÁCS Tibor

In aging societies, the morbidity and mortality of dementia is increasing at a significant rate, thereby imposing burden on healthcare, economy and the society as well. Patients’ and caregivers’ quality of life and life expectancy are greatly determined by the early diagnosis and the initiation of available symptomatic treatments. Cholinesterase inhibitors and memantine have been the cornerstones of Alzheimer’s therapy for approximately two decades and over the years, more and more experience has been gained on their use in non-Alzheimer’s dementias too. The aim of our work was to provide a comprehensive summary about the use of cholinesterase inhibitors and memantine for the treatment of Alzheimer’s and non-Alzheimers’s dementias.

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.