Clinical Neuroscience

[Report]

LEEL-ŐSSY Lóránt

JANUARY 20, 1993

Clinical Neuroscience - 1993;46(01-02)

[Report of the International Society of Neuropathology Executive and General Assembly Council Meeting and the 32nd Scientific Meeting of the Canadian Neuropathologists Meeting]

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Clinical Neuroscience

[Acute neurosurgical management of severe thoraco-lumbar spinal injuries]

ZSOLCZAI Sándor, PENTELÉNYI Tamás, TÚRÓCZY László, VERES Róbert

[Authors show their experiences with up-to-date segmental stabilization methods in the acute neurosurgical management of severe thoraco-lumbar spinal injuries. Among the 134 acute operations during 5 years with at least 1 year follow-up 81 were performed by Fixateur Interne (AO-ASIF, W. Dick), and 53 by angle-stable posterior plate-fixation (Steffee or Eger plates). Reduction, decompression and stabilization were achieved by these instrumentations. Results are evaluated from the points of view of neurological recovery, bony union, restoration of patients comfort and complications. Also the principles of modern management of spine-injured patients, developed through a long evolution in the last decade, are reviewed. It is stated that both segmental stabilization methods were used as routine, and they were suitable for the treatment of the most of severe thoraco-lumbar spinal injuries. Results of these methods are much better than those of the long-rod systems, but on condition that emergency neurosurgical treatment should be done in the first 6-8 hours together with early skilled and competent rehabilitation in a well trained center.]

Clinical Neuroscience

[Relation of acute and long-therm care of the spinal-cord-injured patients in up-to-date management]

KLAUBER András, ZSOLCZAI Sándor, PENTELÉNYI Tamás, MOLNÁR Ferenc

[The acute emergency management of the spinal-cord-injured patients is one of the most important steps in the long-lasting complex treatment which has great significance for the final results. The method of primary neurosurgical operation must be chosen with regard to the special aspects of subsequent rehabilitation. All conditions of emergency surgery have to be assured during the first 6-8 hours. According to the experiences of the last two decades the problems of the management of spinal-cord-injured patients originated in the shortage of financial sources, theoretical concepts and organization, lack of interest and scientific information. Even now there is no spinal-cord-injured center in Hungary which would provide for the complex management including primary neurosurgical treatment and all details up till the end of paraplegic rehabilitation. Primary treatment (either operative or conservative) and rehabilitation are performed in separate departments. Though there is a significant development in emergency spinal surgery during the last years (and it is a pity this can not be seen in rehabilitation in Hungary), it is still an important demand that emergency surgery should be performed with rehabilitation aspects. Until we have no proper financial conditions to organize spinal centers for both emergency and rehabilitation treatment, our most important task is to develop a unified rehabilitational view of all specialists involved in the complex treatment of spine-injured patients.]

Clinical Neuroscience

[Tethered cord syndrome in adults]

VÁRADY Péter, BENOIST György, PÁSZTOR Emil

[The tethered cord syndrome is resulted from the abnormally low position and traction of the conus medullaris in the lumbosacral region, that leads to progressive dysfunction of the caudal spinal segments. It originates from various ectodermal abnormalities, all due to errors of embryonic development in the 3rd-4th weeks. The disorder is manifested by progressive motor, sensory and trophic deficits in the legs and by incontinence. The adult onset is rare. The MRI is the most important procedure for pathoanatomic evaluation. The surgical aim is to stop progressivity and to cure the reversible deficits. We report our six cases and present the data of the literature concluding the caracteristics, the surgical indications and outcome of the tethered cord syndrome in adults.]

Clinical Neuroscience

[Operative treatment of craniocervical instabilities caused by rheumatoid arthritis]

TURÓCZY László, KENÉZ József, VERES Róbert, NAGY Aladár, PÁSZTOR Emil

[We have investigated and operatively treated 30 patients with craniocervical instabilities caused by rheumatoid arthritis. The evaluation of this patient-material seems to be important, because the indications of operations in the different stages of the disease are not completely clear, according to our knowledge. The atlanto-axial instabilities at the beginning stage, when the serious neurologic signs are absent, can be treated with low risk dorsal stabilization, while we believe the advanced instabilities need combined neurosurgical approach for successful treatment. We must emphasize the importance of detailed early diagnostics, and the prevention in neurosurgical approaches, in accordance of the opinions, expressed in different contributions in the literature, dealing with the subject. In 20 cases we had to perform combined surgical interventions, and only in 10 cases, the simple dorsal fixation proved to be satisfactory, which fact shows, that we got patients mainly in more serious stages. We detected a new sign ("ghost tumor") in the natural course of the disease.]

Clinical Neuroscience

[Our experiences in the conservative treatment of the compression fractures of the thoracolumbar spine]

URBÁN Ferenc, TAKÁCS Ferenc

[The authors at the DOTE Department of Traumatology made follow-up examinations of 136 patients treated for compression fractures of the thoracolumbar spine. 63 patients (46,3%) were treated with plaster corset, 73 patients (53,7%) with modified Magnus method. They compared the two methods of treatment for their possibilities and effectiveness. They used plaster corset after reposition in females under 55 years of age and in males under 60 years of age, if the patients' physical and general condition were suitable and the compression was more than 10 degrees. 47,9% of the patients treated functionally recovered well and 31,5% were in a satisfactory state. And 47,6% of the patients treated with plaster corset recovered well and 46,0% recovered satisfactorily. While in the first group i.e.those treated functionally, 17,8% of the patients had severe pain, only 6,4% of the patients treated with corset had similar pain. The authors ascertain that as the anatomical reposition of the fractures is not possible indefinitely, the insertion of the plaster corset when the compression exceeds 10–15 degrees is more suitable. When the deformity decreases, the subjective complaints of the patients decrease too. In the case of small compression, functional treatment is recommended and the patient must lie in bed to alleviate the pain. Both the methods used under appropriate conditions are useful and complement each other. ]

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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

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

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

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

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

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.