Clinical Neuroscience

[Cerebral inorganic and pigment deposits in hallervorden-spatz disease]

KRUTSAY Miklós1, GARZULY Ferenc2

MARCH 20, 1993

Clinical Neuroscience - 1993;46(03-04)

[Authors survey the histochemistry of the cerebral deposits (lipofuscin, ceroid, hemosiderin, ferric phosphate and calcium phosphate) occurring in the Hallervorden-Spatz disease.]

AFFILIATIONS

  1. Ajkai Városi Kórház Pathologiai Osztály
  2. Szombathelyi Megyei Kórház Ideggyógyászati Osztály

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Clinical Neuroscience

[Post-stroke depression]

ASZALÓS Zsuzsa, PATAKY Ilona, SIMON Andrea, NAGY Zoltán

[The pathogenesis of depression following cerebrovascular accident (post-stroke depression) is poorly understood. Thirty seven (28%) out of 134 patients from the „Budapest Stroke Data Bank" were found to be depressed. Thirty two patients (86%) in this group were affected by depression within three months or the stroke. Diagnosis of depression was bades on CES-D, Ham-D and Zung scales, and a word fluency test was performed with 11 depressed and 11 non-depressed patients. Comparing the frequency of post-stroke depression in groups with ischemic damage of the carotid versus the vertebrobasilar system, as well as the left versus right middle cerebral artery, no significant differences were found. Depression reduced the activities of daily living (score: 7.8) compared to the activities of non-depressed patients (score: 3.8) at the same score of stroke – severity (4.7 versus 4.1). These observations suggest that post-stroke depression influences the rehabilitation of stroke patients, therefore the diagnosis and treatment of post-stroke depression may increase the efficacy of stroke rehabilitation.]

Clinical Neuroscience

[The role of brainstem auditory evoked potentials in the diagnosis of vertebrobasilar insufficiency]

MOGYORÓS Ilona

[The diagnostic usefulness of brainstem auditory evoked potentials was compared with other diagnostic possibilities. Brainstem auditory evoked potential was examined in patients with vertebrobasilar insufficiency. Based on the duration of clinical symptoms, patients were divided into three groups: 85 patients with transient ischaemic attack (TIA), 31 patients with prolonged ischaemic symptoms, and 35 patients with chronic symptoms were examined. The latency and interpeak latency of the auditory evoked potentials increased in 49,3 per cent. A subgroup within the TIA group was specially tested. The patients belonging to this group were admitted to the clinic soon after the onset of TIA. Acoustic evoked potentials showed increased latencies and interpeak latencies in 45,8 per cent of this group. The increasing latencies of the waves I. and III. and the interpeak latancies of I-II. and I-V. waves were the most frequent differences. Otoneurology demonstrated more frequent functional disturbances in brainstem than in acoustic evoked potentials in the second and third group. Computerized tomography is a really helpful only in examining chronic cases, while the CT reports 3,6 per cent hypodensities in the first group and 17,3 per cent in the third group. Brainstem auditory evoked potential testing is the most sensitive diagnostic method in the diagnosis of transient ischaemic attack.]

Clinical Neuroscience

[Spontaneous intracerebral haematomas in children]

VELKEY Imre, DOBAI József, GYARMATI János

[The authors report two cases of spontaneous intracerebral haematomas in children. Both children presented with serious symptoms of acute intracerebral haemorrhage. Investigations do not reveal the cause of the haemorrhage in either of the cases and angiography detected no arteriovenous malformation. The term ,,occult” is reserved for these cases, as spontaneous intracerebral haematomas do not result in space-occupying process or CSF passage blockage and may be treated medically.]

Clinical Neuroscience

[The effect of cerebrospinal fluid drainage on middle cerebral artery blood flow velocity in conditions with raised intracarnial pressure]

BARZÓ Pál, DÓCZI Tamás, KOPNICZKY Zsolt, ROTYIS Mária, BODOSI Mihály

[Middle cerebral artery (MCA) blood flow velocity and intracranial pressure (ICP) were recorded in 42 patients suffering from raised ICP. A major (ICP25 mmHg) or moderate (251CP15 mmHg) degree of intracranial hypertension was reduced by means of either continuous or intermittent CSF drainage. Measurements of MCA blood flow velocity were carried out with transcranial Doppler sonography (TCD). Three types of reactions were observed with regard to cerebral blood flow velocity (CBFV) changes in response to CSF drainage. Patients in Group 1 demonstrated pressure passive CBFV changes throughout the observed cerebral perfusion pressure (PP) range. In Group 2, cerebrospinal fluid (CSF) drainage brought about a transitory increase in CBFV for a few minutes. In Group 3, the reduction of ICP to the normal level did not influence CBFV at all. The pulsatility index (PI) of the cerebrovascular reserve capacity (CVR), which was highest in Group 1, changed in contrast with the CBFV changes in Groups 1 and 2. The pressure-passive velocity pattern (in Group 1) suggests that the blood vessels were at nearly maximum dilatation and were perhaps failing to constrict properly in response to increased PP. As recovery proceeded (Group 2), the mechanism became effective, thereby reestablishing autoregulation. Whereas clinical signs and computed tomography reveal only the trend of the ICP, TCD provides the possibility of a semi-quantitative evaluation of ICP changes and seems especially promising in the rapid assessment of the efficacy of treatment aimed at ICP reduction.]

Clinical Neuroscience

[Measurements of regional cerebral blood flow and blood flow velocity in experimental intracarnial hypertension: infusion via the cisterna magna in rabbits]

BARZÓ Pál, DÓCZI Tamás, CSETE Klára, BUZA Zoltán, BODOSI Mihály

[The cerebral blood flow velocity (CBFV), as measured by transcranial Doppler sonography (TCD) via the transorbital route in the intracranial segment of internal carotid artery (ICA), and the regional cerebral blood (volume) flow (rCBF) in the corresponding cortical areas, as measured by the hydrogen clearance technique, were determined in 8 New Zealand White rabbits undergoing cisterna magna infusion for elevation of the intracranial pressure (ICP). In the lower range of autoregulation, i. e. at perfusion pressures (PP) between 80 and 40 mm of mercury and even below this, the changes in (CBFV) and CBF showed a strong correlation (0,86) under conditions with standard PCO2 (PaCO2)=35+2 mm of mercury). Autoregulation was exhausted at 40 mm of mercury and the cerebrovascular resistance (CVR) was minimal. Below this PP, the CBF and CBV dropped sharply, whereas CVR, gradually increased, indicating that, despite the maximally dilated resistance vessels, the intracranial hypertension causes the vascular resistance to increase, possibly via blocking of the venous outflow. Our results confirmed that non-invasive and easily (even at the bedside) applicable measurements of CBFV changes could substitute the cumbersome and expensive isotope measurements of CBF in intracranial hypertension.]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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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

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

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

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

[The role of sleep in the relational memory processes ]

CSÁBI Eszter, ZÁMBÓ Ágnes, PROKECZ Lídia

[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

Clinical Neuroscience

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

EVRAN Sevket, KATAR Salim

Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.