Clinical Neuroscience

[Péter Rajna : Aging brain - aging mind - ageless (?) soul]

KOPP Mária

JANUARY 22, 2008

Clinical Neuroscience - 2008;61(01-02)

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Clinical Neuroscience

[Occlusive hydrocephalus caused by a fourth ventricle arachnoid cyst (in English language)]

SZŰCS Anna, VÁRADY Péter, PESTALITY Péter, FABÓ Dániel, LALIT Narula, KENÉZ József

[The case history of a woman with occlusive hydrocephalus caused by a fourth ventricle cyst is presented. She had slowly progressive complaints and symptoms - concentration and memory disturbances, low-tempered mood, then slight dizziness, loss of appetite and progressive headache - transitorily misinterpreted for signs of depression. She had been treated by psychotherapy and antidepressants for months. Since she did not improve she was referred to a psychiatric hospital. The rapidly progressing neurological syndrome with worsening headache, gait disturbance and vomiting was finally identified and it turned out to be caused by a fourth ventricle CSF blockage of unknown aetiology. An acute neurosurgical intervention was indicated. It revealed a huge fourth ventricle cyst, undetectable on MRI, occupying the whole ventricle. The resection of its walls resulted in complete recovery. We conclude that since unspecific mental complaints and symptoms suggesting depression may be misleading, their organic origin has to be excluded.]

Clinical Neuroscience

[Functional magnetic resonance imaging in neurology (in English language)]

AUER Tibor, SCHWARCZ Attila, HORVÁTH Réka A., BARSI Péter, JANSZKY József

[The present contribution discusses the clinical use of functional MRI (fMRI) and its role in the most common neurological diseases. FMRI was found a reliable and reproducible examination tool resulting in a wide distribution of fMRI methods in presurgical evaluation of epilepsy in determining the relationship of eloquent areas and the epileptic focus. Preliminary data suggest that fMRI using memory paradigms can predict the postoperative memory decline in epilepsy surgery by determining whether a reorganization of memory functions took place. Speech-activated fMRI became the most used tool in determining hemispheric dominance. Visual and senso-motor cortex can also be routinely investigated by fMRI which helps in decision on epilepsy surgery. FMRI combined with EEG is a new diagnostic tool in epilepsy and sleep disorders. FMRI can identify the penumbra after stroke and can provide an additional information on metabolic state of the threatened brain tissue. FMRI has a predictive role in poststroke recovery. In relapsing-remitting MS an adaptive reorganization can be demonstrated by fMRI affecting the visual, motor, and memory systems, despite preserved functional performance. Much more extensive reorganization can be demonstrated in secondary progressive MS. These findings suggest that the different stages of MS are related to different stages of the reorganization and MS becomes progressive when there is no more reserve capacity in the brain for reorganization. FMRI offers the capability of detecting early functional hemodynamic alterations in Alzheimer’s disease before morphological changes. FMRI can be a valuable tool to test and monitor treatment efficacy in AD. FMRI can also provide information about the mechanisms of different therapeutic approaches in Parkinson disorder including drug treatment and deep brain stimulation.]

Clinical Neuroscience

[Complex regional pain syndrome]

EMBEY-ISZTIN Dezső

[Complex regional pain syndrome is one of the frequently topics to be discussed during International Congresses. Three books have been published recently by the IASP Press (1994., 2001., 2005.) The aim of my publications is to draw the attention to this syndrome frequently unrecognized, and to summarize an interesting case report.]

Clinical Neuroscience

[FREQUENCY OF DIFFERENT FORMS OF DEMENTIA IN THE DEPARTMENT OF NEUROPATHOLOGY OF THE HUNGARIAN NATIONAL INSTITUTE OF PSYCHIATRY AND NEUROLOGY DURING A 3-YEAR PERIOD]

KOVÁCS Gábor Géza, KŐVÁRI Viktor, NAGY Zoltán

[Background - Dementia is an increasing problem of the society. The underlying cause of dementia may be difficult to diagnose during life. Only neuropathological examination gives definite diagnosis. Differences in the reported frequency may be related to factors such as the age or gender of subjects with dementia. Materials and methods - In our neuropathology-based study we examined 156 consecutive subjects clinically diagnosed with dementia during a 3-year period. Using histopathological criteria we calculated the frequencies of various disorders causing dementia. We studied the effect of age and gender on these frequencies. Results - Alzheimer’s disease was the most frequent pathologic finding (57.7%) followed by vascular dementia (43%); diffuse Lewy body disease (15.4%); argyrophilic grain dementia (12.1%), various forms of frontotemporal dementia (5.7%); and other (4.5%). The latter comprise prion disease, alcoholic encephalopathy, and hippocampal sclerosis. Mixed pathology was common: concomitant Alzheimer’s disease was present in 41.6% of diffuse Lewy body disease cases and in 49.2% of vascular dementia patients. Pure disease forms are rare: Alzheimer’s disease: 26.3%, vascular dementia: 17.3%, diffuse Lewy body disease: 5.1%, argyrophilic grain dementia: 2.5%. Females were overrepresented among those with Alzheimer’s disease with age at death above 75 years (p <0.02), while males were overrepresented in patients below 75 years with vascular dementia (p <0.05). Conclusions - Our study indicates that the frequency of neurodegenerative dementias is high in the examined patients, but vascular pathology frequently influences the clinical course.]

Clinical Neuroscience

[THE LANDMARKS OF THE MEASUREMENT OF CEREBRAL BLOOD FLOW]

NYÁRY István

[History of the measurement of local cerebral blood flow may cover a period of one and a half centuries. Parallel forthcoming of both theoretical and technical development were the key elements of ensuing progress resulting in the present state, when by the aid of in vivo blood flow and metabolic maps, we can visualize locales of brain functioning and their interconnections. Two theoretical landmarks should be mentioned in this historic process. First, the work of Adolf Fick, as the starter of quantitative measurements in this field, and Seymour Kety’s model of a single, homogenously perfused tissue element. The solution of this model, in the form of Kety’s equation is still fundamental to present day blood flow mapping techniques. Among the numerous investigators over the past years, two Hungarian scientist can be named as major contributors. Kálmán Sántha made substantial studies with continuous registration of local cerebral blood flow by the aid of thermocouples, while Emil Pásztor invented the hydrogen clearance method for the measurement of local cerebral blod flow both in human and in animal studies.]

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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

Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Clinical Neuroscience

Simultaneous subdural, subarachnoideal and intracerebral hAemorrhage after rupture of a peripheral middle cerebral artery aneurysm

BÉRES-MOLNÁR Anna Katalin, FOLYOVICH András, SZLOBODA Péter, SZENDREY-KISS Zsolt, BERECZKI Dániel, BAKOS Mária, VÁRALLYAY György, SZABÓ Huba, NYÁRI István

The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.

Clinical Neuroscience

[Clinical neurophysiological methods in diagnosis and treatment of cerebrovascular diseases]

NAGY Ildikó, FABÓ Dániel

[Neurophysiological methods are gaining ground in the diagnosis and therapy of cerebrovascular disease. While the role of the EEG (electroencephalography) in the diagnosis of post-stroke epilepsy is constant, quantitative EEG para-meters, as new indicators of early efficiency after thrombolysis or in prognosis of patient’s condition have proved their effectiveness in several clinical studies. In intensive care units, continuous EEG monitoring of critically ill patients became part of neurointenzive care protocols. SSEP (somatosesnsory evoked potencial) and EEG performed during carotid endarterectomy, are early indicative intraoperativ neuromonitoring methods of poor outcome. Neurorehabilitation is a newly discovered area of neurophysiology. Clinical studies have demonstrated the effectiveness of repetitive transcranial magnetic stimulation (rTMS) in the rehabilitation of stroke patients. Brain computer interface mark the onset of modern rehabi-litation, where the function deficit is replaced by robotic tehnology. ]

Clinical Neuroscience

[Earlier and more efficiently: the role of deep brain stimulation for parkinson’s disease preserving the working capabilities]

GABRIELLA BALÁS, ISTVÁN KOMOLY, SÁMUEL DÓCZI, TAMÁS JANSZKY, JÓZSEF ASCHERMANN, ZSUZSANNA NAGY, FERENC BOSNYÁK, EDIT KOVÁCS

[Background – The recently published “EarlyStim” study demonstrated that deep brain stimulation (DBS) for the treatment of Parkinson’s disease (PD) with early fluctuations is superior to the optimal pharmacological treatment in improving the quality of life and motor symptoms, and preserving sociocultural position. Our retrospective investigation aimed to evaluate if DBS therapy was able to preserve the working capabilities of our patients. Methods – We reviewed the data of 39 young (<60 years-old) PD patients who underwent subthalamic DBS implantation at University of Pécs and had at least two years follow-up. Patients were categorized into two groups based on their working capabilities: Patients with active job (“Job+” group, n=15) and retired patients (without active job, “Job-” group, n=24). Severity of motor symptoms (UPDRS part 3), quality of life (EQ-5D) and presence of active job were evaluated one and two years after the operation. Results – As far as the severity of motor symptoms were concerned, similar (approximately 50%) improvement was achieved in both groups. However, the postoperative quality of life was significantly better in the Job+ group. Majority (12/15, 80%) of Job+ group members were able to preserve their job two years after the operation. However, only a minimal portion (1/24, 4.2%) of the Job- group members was able to return to the world of active employees (p<0.01, McNemar test). Conclusion – Although our retrospective study has several limitations, our results fit well with the conclusions of “EarlyStim” study. Both of them suggest that with optimal timing of DBS implantation we may preserve the working capabilities of our patients.]