Clinical Neuroscience

[Posterior cortical atrophy (Benson-syndrome)]

RÓZSA Anikó, SZILVÁSSY Ildikó, KOVÁCS Krisztina, BOÓR Krisztina, GÁCS Gyula

JANUARY 30, 2010

Clinical Neuroscience - 2010;63(01-02)

[We present the characteristics of posterior cortical atrophy - a very rare cortical dementia - in a 69 year old woman’s case. Our patient’s symptoms began with a visual problem which was initially explained by ophthalmological disorder. After neurological exam visual agnosia was diagnosed apart from other cognitive disorder (alexia without agraphia, acalculia, prosopagnosia, constructional disorder, clock-time recognition disorder, dressing apraxia, visuospatial disorientation). The brain MRI showed bilateral asymmetric parieto-occipital atrophy which is characteristic of posterior cortical atrophy.]

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[Hungarian Epilepsy League - Account on the meeting of the Hungarian Epilepsy League]

SZUPERA Zoltán

Clinical Neuroscience

[Frontotemporal dementia, Pick’s disease]

ANDREW Kertesz

[A significant expansion of knowledge in the last few years, especially in the molecular biology of frontotemporal dementia (FTD) is summarized. This condition, formerly known as Pick’s disease and considered rare, is estimated to be 12-15% of all dementias and 30-50% early onset ones. The clinical picture is protean, mainly a behavioural and language impairment, but the extrapyramidal syndromes of CBD and PSP also belong. These seemingly different presentations converge, as one or other areas in the brain are affected. Less than half of the cases are tauopathies, the majority has been discovered to have a TDP-43 and most recently a FUS proteinopathy, shared with ALS, opening potential opportunities for pharmacological approaches to treatment. Tau and progranulin mutations on Ch-17 and some others, point to molecular mechanisms. A glossary is provided to navigate the complex terminology.]

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[Congress calendar]

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[Anterior cervical fusion on the lower cervical spine: own clinical experiences]

PAZDERNYIK Szilárd, SÁNDOR László, ELEK Péter, BARZÓ Pál

[Both acute and chronic instability of the cervical spine can be succesfully treated by anterior crevical fusion. The main goal is to create a spondylodesis through which the instable motion segments are fixed in the position defined by the surgeon. The spondylodesis is realised by the bone healing of the intervertebral space. The consolidation itself is facilitated by the operative stabilisation of the segments involved, and also by the implantation/transplantation of the osteoproductive/ osteoinductive materials. The sooner consolidation is achieved, the more likely it is to be able to avoid the material dependent complications and/or that of dislocation. So as to support this theory a retrospective clinical/radiological study was performed. During this the length and the safety of the consolidation was measured by applying various anterior cervical plating systems. A total of 485 patients having cervical injuries or degenerative disc disease were treated by anterior cervical plating. For bone transplantation partly pure autolog spongious partly autolog cortico-spongious morsalised bone chips, furthermore autolog tricortical bone block were applied. A standard protocoll was used for data collection, evaluation and also follow-up. The patients treated with plate systems were divided into 3 groups: Group 1: Non-locked H-plate system with autogeneous cancellous bone (155 trauma patients, for a total of 210 cervical motion segments, 1.35 segments/patients). Group 2: Non-locked H-plate system with tricortical autograft (167 patients, for a total of 290 cervical motion segments, 1.73 segments/patients). Group 3: Locked cervical plate system with tricortical autograft (73 patients, for a total of 110 cervical motion segments, 1.5 segments/patients). Patients treated with standalone cage belong to group 4. These cages were filled with autogenous cortico-spongiosus bone chips (90 patients, for a total of 90 cervical motion segments, 1.0 segments/patients). Evaluations included postoperative clinical, X-ray and CT examination, and follow-ups at 6, 16, 52, and 104 weeks. We established three grades, and classified the degree of bony fusion between the graft and vertebra: not-yet-fused, fused or non-union. When evaluating the results the following statements/observations were made: a) There is a fast and safe consolidation in the case of those patients that underwent dinamic disc osteosynthesis (p=0.00001). b) Whereas performing fixation with non-locked or locked screw plate systems and strutgrafted with tricortical autograft created prolonged healing requiring months and developed non-unions more often (non-locked screw-plate system versus locked screwplate system) (p>0.05). c) Using locked screw-plate fixation systems non-union rate in our study was 21%, suggesting that this form of fixation has only a limited use. d) In our study complete consolidation without pseudoarthrosis was achieved by using standalone cages filled with autolog cortico- spongiosus bone chips, but bony healing was delayed due to cage coating and the substitution of pure autogenous spongiosa for cortico-spongiosus bone chips. It is recommended to treat acute/chronic instability of the cervical spine both by using non-fixed plate system with autolog cancellosus bone and by standalone cage filled with cortico-spongiosus bone chips as well. It is worth keeping in mind that by applying this lattest an extra surgery to harvest the graft will be avoided.]

Clinical Neuroscience

[Molecular genetic mutation analysis in Menkes-disease with prenatal diagnosis]

LÁSZLÓ Aranka, ENDREFFY Emőke, TÜMER Zeynep, HORN Nina, SZABÓ János

[Menkes disease (MD) is an X-linked recessive multisystemic lethal, heredodegenerative disorder. Progressive neurodegeneration and connective tissue disturbances with microscopically kinky hair are the main symptoms. Molecular genetic mutation analysis was made at a Hungarian male infant suffering from MD and prenatal diagnosis was done in this MD loaded family. Method - The 12th exon of ATP7A gene has been analyzed by dideoxy-finger printing (DDF), polymerase chain reaction (PCR), direct sequencing of exon 12. The specific mutation was screened from chorionic villi of the maternal aunt at the 14th gestational week. Results - In the exon 12th a basic pair substitution with Arg 844 His change was detected leading to very severe fatal missense mutation.]

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

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Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

The etiology and age-related properties of patients with delirium in coronary intensive care unit and its effects on inhospital and follow up prognosis

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Delirium is a syndrome frequently encountered in intensive care and associated with a poor prognosis. Intensive care delirium is mostly based on general and palliative intensive care data in the literature. In this study, we aimed to investigate the incidence of delirium in coronary intensive care unit (CICU), related factors, its relationship with inhospital and follow up prognosis, incidence of age-related delirium and its effect on outcomes. This study was conducted with patients hospitalized in CICU of a tertiary university hospital between 01 August 2017 and 01 August 2018. Files of all patients were examined in details, and demographic, clinic and laboratory parameters were recorded. Patients confirmed with psychiatry consultation were included in the groups of patients who developed delirium. Patients were divided into groups with and without delirium developed, and baseline features, inhospital and follow up prognoses were investigated. In addition, patients were divided into four groups as <65 years old, 65-75 yo, 75-84 yo and> 85 yo, and the incidence of delirium, related factors and prognoses were compared among these groups. A total of 1108 patients (mean age: 64.4 ± 13.9 years; 66% men) who were followed in the intensive care unit with variable indications were included in the study. Of all patients 11.1% developed delirium in the CICU. Patients who developed delirium were older, comorbidities were more frequent, and these patients showed increased inflammation findings, and significant increase in inhospital mortality compared to those who did not develop delirium (p<0.05). At median 9-month follow up period, rehospitalization, reinfarction, cognitive dysfunction, initiation of psychiatric therapy and mortality were significantly higher in the delirium group (p<0.05). When patients who developed delirium were divided into four groups by age and analyzed, incidence of delirium and mortality rate in delirium group were significantly increased by age (p<0.05). Development of delirium in coronary intensive care unit is associated with increased inhospital and follow up morbidity and mortality. Delirium is more commonly seen in geriatric patients and those with comorbidity, and is associated with a poorer prognosis. High-risk patients should be more carefully monitored for the risk of delirium.

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

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Clinical Neuroscience

To handle the HaNDL syndrome through a case: The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis

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The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related to migrainous headaches. It is a benign, self-limited disorder, which is characterized by fluctuating neurological symptoms and cerebrospinal fluid lymphocytosis. We describe a case of a 47 years old man with acute onset of headache and aphasia. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis (25 cells/μl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with temporoparietal predominance, and without epileptiform activity. His blood tests as well as magnetic resonance imaging (MRI) results were normal. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged with full recovery.

Clinical Neuroscience

[Interdisciplinary approach of vestibular system impairment]

PONGRÁCZ Endre

[In the first part of this review the definition of vertigo/dizziness was discussed. The major difference between the two signs is the exsistence of the direction, which is specific for vertigo. Dizziness is a frequent complaint in the clinical practice. Its frequency is increasing with advance of age, to intimate the play of declining cognitive process in the pathogenesis of its. The popular health significance of vertigo is in the rowing number of the patients. The onset of the most cases with acute vertigo appears between secundums and minutes so the patients will be provided in circumstances of emergency department. First of all three form schould be take into account: neuronitis vestibularis, benign paroxysmal positional vertigo and Meniere syndrome. Without tipical periferal signs of vertigo, central cause should be searched, principally stroke (lysis possibility). The differential diagnose of the different dizzeness/vertigo forms according to the elapsed time of the onset or congenital and acquired nystagmus was created in tables. The recommendations of the therapy of acute and chronic dizziness/ vertigo syndroms are, lack of results of evidence based trials doubtful. The more often used drugs based on clinical trials are discussed as vinpocetine, betahistine and piracetam. The in vitro and in vivo data suggest that the last molecule is eligible to use both in periferal and central type of vertigo syndroms.]