Clinical Neuroscience

[Spiractin electroencephalography tests]

SOMOGYI István1

DECEMBER 01, 1961

Clinical Neuroscience - 1961;14(12)

[Studies with Spiractin have shown that: 1. in addition to its effect on the respiratory center, it has an excitatory effect on the diffuse activating system of the brainstem. 2. it can be used as a provoking agent in electroencephalography of patients with epilepsy (especially in children). 3. It may also occasionally cause generalized seizures, which should be kept in mind in cases of epilepsy.]

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  1. Szegedi Orvostudományi Egyetem Ideg-Elmeklinikája

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Clinical Neuroscience

Entwicklung – Reaktion — Prozess

NIKOLA Schipkowensky

In dieser Begriffsbestimmung wird die Untrennbarkeit des somatischen und psy chischen Lebens in den Vordergrund gestellt, was allerdings nicht besagen will, sie seien identisch. Schon dadurch ist aber unterstrichen, dass die Materie das Primäre ist, der Geist — das Sekundäre ; denn Form ohne Inhalt ist undenkbar. Das Bewusstsein, als höchste Form menschlichen Daseins, darf man nicht als ein blosses Epiphänomen seines Organs hinstellen. Die psychische Tätigkeit wird zwar vom Grosshirn geleistet, sie ist aber in ihren Inhalten und in ihrer logischen Gliederung von der objektiven Wirklichkeit deter miniert, deren subjektive Abbildung sie darstellt. Die Definition verweist ferner auf den biologischen Ursprung der menschlichen Persönlichkeit, deren weitere Entwicklung auf der Verbindung von unbedingten Reflexen mit der Er arbeitung kompliziertester dynamischer Stereotypen zeitlicher Verknüpfungen beruht. Schliesslich setzt diese Definition auch die Untrennbarkeit von Psychi atrie und Neurologie voraus : Sie sind einheitliche Glieder der einen medizi nischen Wissenschaft.

Clinical Neuroscience

[On surgical indications for metastases of spinal carcinoma]

PÁSZTOR Emil, NAGY Vilmos

[Based on our experience with 40 patients with spinal metastases, we do not refrain from operating on single nodal spinal metastases and paraplegia alone is not a contraindication. The indication for surgery and the expected results depend mainly on the histological and biological characteristics of the primary tumour, the course of the disease and the general physical condition of the patient. Based on the catamnestic evaluation of operated and non-operated patients, surgery can be recommended. Since the severity of compression or paresis is significant in the postoperative outcome, early surgery is desirable. ]

Clinical Neuroscience

[Multiple sclerosis and generalised reticulosarcoma combined case]

PAPP Mátyás, KIRÁLY Ferenc

[A 63-year-old patient had multiple sclerosis for about 20 years, which progressed in relapses. 8 months before his death, he developed generalized lymph node enlargement. Autopsy and histopathological examination showed generalized reticulosarcoma throughout the body and diffuse soft tissue and perivascular tumour proliferation in the central nervous system. With minor exceptions, the pia-glia membrane inhibited spread into the neural tissue everywhere except in the corpus pinealea, which may be attributed to the specific biological behaviour of the corpus pinealea in the face of pathological processes. In the central nervous system, in addition to the tumoral proliferation occupying the perivascular spaces, we found multinodular marrow neoplasms, which correspond in their location and histological features to old multiple sclerosis foci. On the basis of the histological picture and the clinical course, the described case must be considered as an accidental encounter of two separate diseases. ]

Clinical Neuroscience

[Rare symptom of cervical vertebral chordoma]

HAFFNER Zsolt, DÖMÖTÖR László, VACZÓ György

[A 60-year-old man with a cervical chordoma, the clinical picture of which was fluctuant and the diagnosis of which could only be clarified by histopathological examination. Contrary to previous literature, reactive marginal sclerosis was detected in vertebra C III, with the chordoma as the primary cause. This case confirms the phenomenon that the clinical picture of vertebral chordoma is atypical and thus difficult to diagnose clinically. ]

Clinical Neuroscience

[Report on the EEG training course in Marseille ]

WALSO Róbert

[The author reports on the EEG course in Marseille, organized by the International Federation of Societies for EEG and Clin. Neurophysiology" in Marseille from 28 August to 2 September 1961.]

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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.

Clinical Neuroscience

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

NALBANTOGLU Mecbure, AKALIN Ali Mehmet, GUNDUZ Aysegul, KIZILTAN Meral

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

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

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.

Lege Artis Medicinae

[Current issues in the peripartum management of diabetic women from the perspective of an internist-diabetologist]

KERÉNYI Zsuzsanna

[In pregnancy complicated with diabetes, treatment of hyperglycaemia is of fundamental importance during delivery in order to improve the outcome parameters of both the mother and the neonate. This is particularly important in the case of mothers with type 1 diabetes and of all mothers who require insulin treatment during their pregnancy. The use of antenatal steroids for women at risk of pre-term birth further complicates the treatment of hyperglycaemia in the period immediately before delivery and requires the appropriate change of insulin therapy. The requirement of nil per os in the delivery period necessitates proper fluid, glucose and insulin treatment in the pre-delivery hours. After surgical delivery the patients may also need infusion treatment until the first meal. As there is no unified guideline for the peripartum management of diabetes, the author re­views the international literature on the internal medicine issues concerning the peripartum treatment of pregnant women with diabetes. This study reviews the characteristics of insulin treatment of women with various types of diabetes before, du­ring and di­rect­ly after delivery. It presents a dosing schedule for women who needed an antenatal steroid treatment in the period before delivery due to premature birth for the purpose of lung maturation. The study also addresses the application and programming of pe­ripartum blood glucose tests, continuous interstitial glucose monitoring (CGM) and insulin pump treatment (CSII).]