Clinical Neuroscience

[Vascular compression syndromes of the cranial nerves]

KUNCZ Ádám, VÖRÖS Erika, BARZÓ Pál

JANUARY 30, 2011

Clinical Neuroscience - 2011;64(01-02)

[The blood vessels which are running nearby the cranial nerves and the brainstem can be elongated; curves and loops of the vessels may develop mostly due to the degenerative alterations of ageing and these vessels can compress the surrounding neural elements. The authors report a review of vascular compression syndromes based on the literature and their own experience. The typical clinical symptoms of the syndromes subserving the proper diagnosis, the pathomechanism, the significance of imaging especially the magnetic resonance angiography, the experience with the surgical technique of microvascular decompression which is the only causal treatment of the syndromes are discussed. In cases of non-responsible medical treatment the microvascular decompression should be the eligible treatment in certain syndromes (trigeminal and glossopharyngeal neuralgia, hemifacial spasm) for it is a highly effective and low risk method.]

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Clinical Neuroscience

[Ulcerative carpal tunnel syndrome]

KAMONDI Anita, TEIXEIRA Jose Maria, SZIRMAI Imre

[The carpal tunnel syndrome is the most frequent compression-induced neuropathy. A severe but rare clinical manifestation of this disorder associates with ulceration, acral osteo-lysis and mutilation of the terminal phalanges of the second and third fingers. Recognition of this disorder is difficult, because various dermatological and internal diseases might lead to acral ulcerative lesions, and these patients are seldom referred to neurological and/or electrodiagnostic examination. In this article, we present three cases of this rare clinical form of carpal tunnel syndrome and discuss the electrodiagnostic findings. The early diagnosis is important since decompression of the median nerve in due time might prevent mutilation and could significantly improve the patients’ quality of life.]

Clinical Neuroscience

[EEG investigations in cognitive impairments]

SZIRMAI Imre, KAMONDI Anita

[The EEG is an indicator of all physiological and neuropsychological activity. The α rhythm was considered as a key phenomenon in research of human mentation from the discovery of EEG. Two methods are known for the estimation of cognitive deficit by the use of quantitative EEG (QEEG). The first is based on the hypothesis, that the mean values of the normal EEG from healthy volunters can be used as reference, and deviation from the normal values of EEG parameters may suggest disease. This kind of “neurometry” was elaborated by R. E. John. The second method asseses event related (ER) transients evoked by somatosensory and mental stimuli. Quantity and localization of signals may refer to the functional state of the cortex. These reactions depend strongly on the test-paradigms. Recognition of the attention-intention cycle disclosed the psysiological mechanism of ERD (event related desynchronisation) and ERS (event related synchronisation). In contrast with the classical “stimulus-reaction” model, both perception and voluntary movement are iniciated by the brain itself, and not by the environment. Human behavior and conscious actions depend on the intention. QEEG analysis proved that the attention and intention localize in segregate areas of the brain. Both “static” and “dinamic” neurometric methods are able to differenciate the EEG records of demented patients from healthy controls, furthermore some dementias from each other. We conclude that with the help of sofisticated methods of QEEG analysis minimal functional deficit of the electrogenesis can be recognized, which could be helpful in the differential diagnosis Notwithstanding the EEG can not explain the evolution neither the normal or the diseased mental processes. The only “instrument” which is able to approach the human mind is the human cogitation itself with the aids of appropriate tests. The QEEG can be conclusive in the analysis of particular processes of mental activity, such as timing, state of activation, hierarchical organisation of cortical territories and mechanism of electrogenesis.]

Clinical Neuroscience

[Neurological complications of Fabry-disease]

VASTAGH Ildikó, CONSTANTIN Tamás, KÉRI Anna, RUDAS Gábor, FEKETE György, BERECZKI Dániel

[Background - Fabry-disease (FD) is a rare X-linked lysosomal storage disease. Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, myocardium and in ganglions of the peripheral and autonomic nervous system and causes diverse symptoms. The classical phenotype is seen in most males and rarely in females. In women, symptomes start later and the severity is milder. Both peripheral and central nervous system can be both affected. Objectives - Fabry-patiens and gene-carriers in the central region of Hungary are treated in the 2nd Pediatric Department, Semmelweis University. These patients are consulted by an interdisciplinary team. At present, four hemizygous male, four heterozygous female Fabry-patients and three asymptomatic heterozygous gene carriers are followed. Results - After reviewing the neurological complications of FD, we present clinical and neuroimaging data of our patients. Conclusion - We emphasize that neurologists should suspect the rare monogenic FD in the case of acroparaesthesia and heat-cold intolerance in childhood or adolescence; clinical signs of TIA/stroke or unexplained MRI alterations suggesting small vessel disease in young adults. Early diagnosis and introduction of enzyme replacement therapy (ERT) can halt or reverse progression.]

Clinical Neuroscience

[Tolosa-Hunt syndrome]

PÉTERFI Anna, ZÁDORI Péter, SÜTŐ Gábor, HORVÁTH Gyula, KOPA János

[Both men and women are affected by the rare disease called Tolosa-Hunt syndrome. We don’t know exactly what causes it to evolve. It is usually put into the categories of either idiopathic inflammation or pseudotumor. Its patological feature is a non-specific inflammatory process with fibroblastic, lymphocytic, plasmocytic infiltration, which can be found, for the most part, in the wall of the sinus cavernosus. Granulocytic and giant-cell infiltrations have been described too. The possibility of autoimmune disease has also come up. In our current study we describe the case of a female patient who recovered with the help of a steroid therapy. Through examining her, we also found immunological alterations, which should urge us to thoroughly examine the further observations of this kind.]

Clinical Neuroscience

[Paroxysmal kinesigenic dyskinesia]

VADÁSZ Dávid, KLIVÉNYI Péter, VÉCSEI László

[Paroxysmal kinesigenic dyskinesia (PKD) is a rare neurological disease and the diagnosis is based on case history and clinical features. Despite of simply diagnostic criteria, the recognition of the disease is sometimes delayed. The involuntary movements in PKD lead to anxiety, social isolation, trauma and worsens the quality of life. To establish the diagnosis many other paroxysmal syndromes have to be excluded. The disease responds to antiepileptic therapy well. The genetic background of the familiar cases is not known. Here we present a 19 year-old patient with PKD and review the current literature. Our patient’s events were triggered by sudden movements and last several seconds. His physical and neurological examinations were normal and responded well to carbamazepine therapy.]

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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.

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Acute effect of sphenopalatine ganglion block with lidocaine in a patient with SUNCT

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Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

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.

Clinical Neuroscience

CANOMAD syndrome with respiratory failure

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CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.