Clinical Neuroscience

[Cerebellar venous anomalies with symptomatic vascular malformations]

KOVÁCS Tibor, PAJOR Péter, BODROGI László, FARSANG Marianna, JUHÁSZ Csaba, SZIRMAI Imre

AUGUST 20, 2002

Clinical Neuroscience - 2002;55(07-08)

[Cerebral and cerebellar venous anomalies (previously known as venous angiomas) form the alternative venous drainage of the surrounding nervous tissue because of the un-development of the normal venous system. They are made up of veins with abnormal structure: thick walls, lumens dilated and of irregular calibre that converge radially towards a wide draining vein (caput medusae). They are thought to be a benign condition although they are sometimes associated with cerebellar hemorrhages. Authors report three patients with cerebellar venous anomalies associated either with pontine cavernoma, cerebellar arteriovenous malformation or cerebellar infarct. They illustrate that cerebellar venous anomalies are benign conditions, but their presence might be a marker for additional, pathogenic malformation. It might be difficult to detect the associated malformations even by sophisticated imaging methods, but their presence can modify the treatment options.]

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Clinical Neuroscience

[The molecular genetic control of bony developmental malformations affecting the craniocervical junction and the cervical spine]

DÁVID Károly, KASÓ Gábor, THOROGOOD Peter V, STEVENS John M, CROCKARD H Alan

[In this review a new interpretation of the origin of bony developmental malformations affecting the craniocervical junction and the cervical spine is presented based on recent advances in the understanding of embryonic development of the spine and its molecular genetic control. Radiographs, CT and MRI scans or CT myelograms of patients with Klippel-Feil syndrome were used for demonstration. Detailed clinical and radiologial analysis of these patients was published earlier [David KM, Stevens JM, Thorogood P, Crockard HA. The dysmorphic cervical spine in Klippel-Feil syndrome: interpretations from developmental biology. Neurosurg Focus 1999;6(6):1.]. Homeotic transformation due to mutations or disturbed expression of Hox genes is a possible mechanism responsible for C1 assimilation. Notochordal defects and/or signalling problems, that result in reduced or impaired Pax-1 gene expression, may underlie vertebral fusions. This, together with asymmetrical distribution of paraxial mesoderm cells and a possible lack of communication across the embryonic mid-line, could cause the asymmetrical fusion patterns. The wide and flattened shape of the fused vertebral bodies, their resemblance to the embryonic cartilaginous vertebrae and the process of progressive bony fusion with age suggest that the fusions occur before or, at the latest, during chondrification of vertebrae. The authors suggest that the aforementioned mechanisms are likely to be, at least in part, responsible for the origin of the bony developmental malformations affecting the craniocervical junction and the cervical spine.]

Clinical Neuroscience

[Application of kinematic parameters for the assessment of impairments due to central motoneuron damage]

FAZEKAS Gábor, FEHÉR Miklós, KOCSIS László, STEFANIK Györgyi, BOROS Zsuzsanna, JURÁK Mihály

[Evidence based medicine requires objective methods for the assessment of status of the patients. The method described by the authors makes it possible to assess motoric impairment of patients in an objective way. It is based on three-dimensional motion analysis. Authors present the case history of two patients with spastic hemiparesis due to central nervous system damage. Changes in motoric impairment were followed by three-dimensional motion analysis. This method can be adapted for the assessment of motor impairment arising from other reasons as well.]

Clinical Neuroscience

[The evolution of psychoneuroimmunology]

SOMOGYI István, SZEKERES György, SZENDI István

Clinical Neuroscience

[Percutaneous procedure for treatment of diseased vertebral bodies with different etiology: vertebroplasty]

KASÓ Gábor, STEFANITS János, KÖVÉR Ferenc, HUDVÁGNER Sándor, DÓCZI Tamás

[Percutaneous vertebroplasty is a radiologically guided invasive technique consisting of the injection of surgical cement into the diseased vertebral body. The procedure results in immediate pain relief and strengthening of the bone due to the polymerization process of the filling material hardening the vertebral body and preventing further collapse. This method is suitable for the treatment of osteoporotic vertebral fractures and of osteolytic vertebral body metastases without neurological signs, in multiple appearance as well. Authors present technical details of the procedure performed by bi-directional fluoroscopy and combined CT-fluoroscopy control as well as short-term experience obtained by treatment of 17 patients.]

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Clinical Neuroscience

[Multiple ischemic stroke in Osler-Rendu-Weber disease]

SALAMON András, FARAGÓ Péter, NÉMETH Viola Luca, SZÉPFALUSI Noémi, HORVÁTH Emese, VASS Andrea, BERECZKY Zsuzsanna, TAJTI János, VÉCSEI László, KLIVÉNYI Péter, ZÁDORI Dénes

[Hereditary hemorrhagic teleangiectasia (HHT, Osler-Rendu-Weber disease) is an autosomal dominantly inherited disorder caused by the mutation of several possible genes and characterized by malformations of the arteriovenous system in multiple organs. The clinical diagnosis is based on the Curaçao criteria ((1) spontaneous, recurrent epistaxis; (2) teleangiectasias in characteristic sites (lips, oral cavity, nose, fingers); (3) visceral lesions (gastrointestinal, pulmonary, cerebral, spinal); (4) affected first degree relative). The aim of this study is to present the first genetically confirmed Hungarian case of hereditary hemorrhagic teleangiectasia with multiple ischemic strokes. Our 70-year-old woman has been suffering from severe epistaxis since her childhood and presented gastrointestinal bleeding during her adulthood as well. The characteristic skin lesions developed in the 5th decade of life. She was admitted to our department with loss of consciousness and fluctuating speech and swallowing problems. MRI of the brain supplemented with angiography revealed multiple arteriovenous malformations and multiple subacute ischemic lesions. The EEG demonstrated slowing of electric activity in the left frontal lobe. The neuropsychological assessment showed deficits in anterograde memory and executive functions. The diagnostic work-up for other characteristic alterations identified an arteriovenous malformation in the left lung. The genetic analysis demonstrated a heterozygous mutation in the 7th exon of the ENG gene at position 834 resulting in a thymine duplication and an early stop codon by a frame shift. The present case is largely similar to those already described in literature and draws the attention to the importance of multidisciplinary collaboration in the care of HHT patients.]

Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

Coexistence of cervical vertebral scalloping, pedicle deficiencies and dural ectasia in type I neurofibromatosis

YALDIZ Mahizer

Neurofibromatosis type 1 (NF-1; also known as Von Recklinghausen’s disease) is a common autosomal dominant disease that occurs in the general population at the rate of 1 in 3000. Many NF-1 patients present with spinal malformations. A 54-year-old female patient was admitted to the Outpatient Clinic of Dermatology with gradually increasing swelling and spots on the body that had been present for a long period of time. Cervical vertebral scalloping, pedicle deficiencies and dural ectasia (DE) were also detected. She was diagnosed with NF-1. NF-1 is routinely seen in dermatology practice. Coexistence of NF-1 with vertebral scalloping, pedicle deficiencies and DE rarely occurs. Our case is the second reported instance in the literature of NF-1 with a spinal anomaly in the cervical region, and the first reported instance of the coexistence of NF-1 with cervical vertebral scalloping, pedicle deficiencies and DE.

Clinical Neuroscience

The applications of transcranial Doppler in ischemic stroke

FINNSDÓTTIR Herdis, SZEGEDI István, OLÁH László, CSIBA László

Background: This overview provides a summary of the applications of transcranial Doppler (TCD) in ischemic stroke. Results: A fast-track neurovascular ultrasound protocol has been developed for detecting occlusion or stenosis. The technique is more reliable in the carotid area than in the posterior circulation. By monitoring the pulsatility index the in­crea­sed intracranial pressure can be diagnosed. TIBI score was developed for grading residual flow. TCD has been shown to accurately predict complete or any recanalization. Regarding recanalization, TCD has a sensitivity of 92%, a specificity of 88%, a positive predictive value of 96%, a negative predictive value of 78% and an overall accuracy of 91%, respectively. Sonothrombolysis seemed to be a promising application but randomized controlled trials have shown that it does not improve clinical outcome. TCD examination can detect microembolic signals (MES) which are associated with an increased risk of stroke. Micro­em­boli were detected in symptomatic and asymptomatic carotid artery stenosis and during carotid endarterectomy. The number of microemboli can be decreased by antithrombotic therapy. Contrast en­chan­ced examination and Valsalva maneuver with continuous TCD monitoring can accurately screen for right-to-left shunt.