Clinical Neuroscience

[Diastematomyelia a rare dysraphic lesion of the spine]

KISS Marianna1, BUZA Zoltán2, VÖRÖS Erika1

SEPTEMBER 20, 1997

Clinical Neuroscience - 1997;50(09-10)

[A split cord malformation may be either diastematomyelia or diplomyelia. The former is characterized by two dural sacs and an osseous or osseocartilaginous septum, while the latter has two hemicords within a single dural sac and a nonrigid septum. Consequently, both require a specific neurosurgical technique. The featured case emphasizes the importance of radiological investigations in the differential diagnosis of these rare entities. The 25 year old woman experienced progressive worsening of sensation of her left foot, lower leg atrophy on the same side and gait disturbance. The findings upon physical examination were slight weakness of the left peroneal muscles, diminished touch sensation over the left foot and position sense in the left toes, and an absent ankle jerk reflex. SSEP of the left lower extremity yielded 3-5 ms P40 peak latency. The plain X-ray films and CT images revealed multiple congenital osseous anomalies: scoliosis, lumbalization of s 1 vertebra, hemiblock of the L 1-3 vertebral bodies, spina bifida at the Th 4-5, L 2-5 and S 1 and a bony median septum at L 2-3. The Il and T2 weighted axial, sagittal and coronal MR images showed sagittal spinal cord cleft with asymmetrical hemicords at L 2-4, and rejoining of them below 14. Low position of the conus, tethered cord and lipomatous infiltration of the filum were also detected. The earlier the detection of a split cord malformation, the better the surgical outcome. From the technical point of view, the preoperative classification with CT and MR is paramount.]

AFFILIATIONS

  1. Szent-Györgyi Albert Orvostudományi Egyetem, Radiológiai Klinika
  2. Idegsebészeti Klinika, Szeged

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Clinical Neuroscience

Management of cavernous angiomas of eloquent brain areas by means of image (MRI or CT) - guided key-hole craniotomy

KÖVÉR Ferenc, DÓCZI Tamás, VON JAKO C., BALÁS István

Details of neurosurgical management of four patients suffering from intractable epilepsy caused by cavernous angiomas of subcortical eloquent brain areas are presented. Two of four cavernomas were not visualized by CT or angiography but only by MRI. MRI or CT stereotactic image-guided key-hole craniotomy and resection of the lesion from the dominant anterior central gyrus (2 cases), from the dominant superior temporal gyrus or from the dominant supramarginal gyrus were performed without any morbidity. The postoperative hospital stay was 3-5 days and all four patients experienced improved seizure control. We conclude that stereotactic image-guided key-hole craniotomy and microsurgical resection offer significant advantages in the treatment of cavernous malformations. Surgical indications include medically refractory epilepsy, intracerebral haemorrhage and/or progressive neurological deficit.

Clinical Neuroscience

[Examination after the first epileptic seizure]

RÓZSAVÖLGYI Margit

[106 patients were examined after the first epileptic seizure; they were divided into two groups based on the clinical data. The two groups were: the group of provoked seizure and the group of non-provoked (isolated) seizure. 95 patients were in these two groups. The others showed focal epileptiform EEG alterations or generalized epileptiform activity without seizures and, because of the very low number of patients, the author excluded them from the study. A questionnaire was used to study and to search for the history of the disease and provocative factors. The familial occurrence of epilepsy was quite often among the patients, similar to primary generali zed epilepsy (17.9%). There were no important previous illnesses, except the provoked seizure. The provocative factors played a significant role in the occurrence of the first seizure. The different provocative factors appeared in greater numbers in the different groups. The anxiety occurred as a possible provocative factor in the group of non provoked seizure. The EEG after the first epileptic seizure showed epileptiform activity in the lower percentage than was predictable from the literature. The mobile long term EEG was shown to be an effective method of examination for epileptiform activity.]

Clinical Neuroscience

[Interpretation of the resoults of DNA image analysis in astrocytomas - biology and prognosis]

SZŰCS István, LEEL-ŐSSY Lóránt, KINDLER Miklós

[Ploidy and proliferative activity of 72 astrocytomas (except glioblastomas) were examined with nuclear DNA content by computed image analysis. The majority of samples originated from paraffin embedded material but they were partly obtained from surgical biopsy by printing or smear technique. The 72 astrocytomas were classified according to Kernohan's grading system. Different areas of tumours were evaluated according to their ploidy (euploid, diploid, aneuploid and heterogenous ranges), proliferative parameters and DNA indices. The transitional zone sometimes revealed higher proliferative index with euploidy. The DNA distribution showed greater population even in the case of moderate malignancy. Uniform – single population - DNA distribution characterized the benign type in 79% with DNA index within the diploid range. The remaining 21% had aneuploidy with another population (heterogenous). The higher the malignancy the greater the population may be found with the higher percentage of S phase cells. The mitotic forms displayed higher DNA index as well as G 2 phase even in the transitional zone of the tumour. The heterogeneity with higher percentage of S- phase correlated well with the histological type of the tumour. The follow-up study of some astrocytomas sometimes did not show a good correlation with the DNA values. However a good correlation was found between the number of cells with higher S and G 2 phases and the type of histogram in the majority of cases studied. The different parts of the tumours also varied in these respects, which should be taken into consideration during needle biopsies.]

Clinical Neuroscience

[Suggestion for new classification of ischemic stroke]

KOPA János, RÁDAI Ferenc, SZÁSZ Krisztina, REPA Imre, HUSZÁR Péter, BESZTERCZÁN Péter, GYŐRBÍRÓ Zsolt

[The classifications of ischemic damage of the brain which have been in use cannot give a suitable orientation about the pathoanatomical background of this disease. As a result of this, the authors investigated the data of 1000 cerebrovascular indoor stroke patients who had been investigated by CT or MRI. The clinical and radiological data were compared. Their conclusion was: more than 80% of the cerebrovascular diseases are ischemic lesions. All of the ischemic patients have some vascular disturbances. In the cases of all patients having some ischemic event (transient ischemic attack, reversible ischemic neurological deficit, prolonged reversible ischemic neurological deficit, completed stroke) there are enduring ischemic lesions which can be demonstrated by CT or MRI. Comparing the neurological signs and the CT or MRI findings of the patients we could establish two groups of ischemic lesions: solitaire and multiplex infarcts. They can be divided into 3 subgroups. Among the solitaire infarcts is subgroup S-1: the patient has no neurological sign and in the CT or MR pictures there is only one lesion under 10 mm in diameter. S-2: mild neurological signs with one infarcted lesion about 15 mm in diameter; as a partial lesion in the territory of a cerebral vessel; S-3: severe neurological signs with total or almost total damage in the territory of a cerebral vessel. Among the multiple infarcted patients there is subgroup M-1: the patient has no neurological sign, but in the CT or MR pictures there are many small (under 5 mm in diameter) infarcted zones; M-2: the patient has mild neurological signs and multiple infarcted zones (5-15 mm in diameter) can be seen in the CT, MRI picture; M-3: the patient has very severe neurological signs and/or dementia. The infarcted areas consist of small and one or more sites of partial or total damage in the territory of a cerebral vessel. This classification is better in showing the state of the patients and the pathoanatomical distur bances. The authors have used this classification in clinical practice without any problems for a year.]

Clinical Neuroscience

Analysis of IQ and genotype in duchenne and becker muscular dystrophy

LÁSZLÓ Aranka, PÓR Erzsébet, CSEPREGI Zsuzsa, ENDERFFY Emőke, RASKÓ István

The authors analysed the association of genotype and the intelligence quotient (IQ) examined with age dependent psychological methods (Wechsler, Vineland, Binet, Raven IQ tests) in 41 Duchenne (DMD), 2 intermediate MD, and 14 Becker type muscular dystrophic (BMD) patients (mean age2.5-37 y). ln 61.4% of the DMD/BMD exon deletion of dystrophin gene was found, while in 38.6% no deletion was detected. The assessment of the genotype was made by using multiple PCR method, examining the most frequent deletion of 18 exons and the muscle specific promoter. Among the deletion patients the distribution of mentol retardation was 28.5%, among patients with non deletion 22.7%. ln 21.4% of BMD patients and in 29.2% DMD patients mentol retardation was detected, the differences were not significant. Similar to the findings of other authors in 4 patients having exon deletions near the 5' end of the gene no mentol retardation was found, but in 14 of 30 patients having deletions near the 5' end of the gene there was. One patient with the largest deletion (exons 12-44) proved to be debil.

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Clinical Neuroscience

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

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

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.

Clinical Neuroscience

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.