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Clinical Neuroscience

MARCH 30, 2020

[Diseases with peripheral motor symptoms ]

DELI Gabriella, KOMÁROMY Hedvig, PÁL Endre, PFUND Zoltán

[Diseases with peripheral motor symptoms are a rare, but important subgroup of the all peripheral neuropathies, radiculopathies and neuronopathies. In these mostly progressive neuropathies, the clinical features include pure motor symptoms with weakness and wasting of the striated muscles. The differentiation of these diseases is frequently a challenge for qualified clinical neurologists. A careful history taking, the disease time course, the findings of routine clinical physical examination and the electrophysiological studies are all necessary in the diagnostic procedure. The aim of this publication is to overview the clinical characteristics of the pure motor peripheral neuropathies, to consider the diagnostic steps and the differential diagnosis, and finally to summarize the treatment options. ]

Clinical Neuroscience

JANUARY 30, 2015

[Assessment of severity and time course of critical illness neuropathy in septic patients: a prospective observational study]

NEMES Réka, FÜLEP Zoltán, LÁSZLÓ István, SÁRKÁNY Péter, FEKETE Klára, MECHLER Ferenc, FÜLESDI Béla

[Objective - In this prospective observational study we investigated electrophysiological alterations in the early phase of critical illness and correlated electrophysiological findings with the clinical picture and outcome. Methods - We enrolled 21 critically ill surgical patients having ≥12 Acute Physiology and Chronic Health Evaluation (APACHE) II scores on admission. Routine non-invasive bilateral electroneurography (ENG) examination of median and ulnar nerves was done on five consecutive days starting in two days after admission. Then weekly follow-up was performed. Motor and sensory nerve conduction indices were calculated and correlated with APACHE II and Simplified Acute Physiology Score II severity scores. Results - On the first examination 18/21 patients had >20% reduction in the motor and sensory nerve conduction indices. Severity score systems showed significant negative correlation with the daily change of CMAP and SNAP amplitudes and calculated nerve conduction indices (Spearman’s correlation, p<0,001). Mortality was higher in the patients with worse admission ENG and/or stagnant electrophysiological status or declining tendency in the first week. Conclusions - Electrophysiological alterations appeared soon after the development of critical illness. Early phase alterations showed a strong correlation with patients’ general condition and more severe electrophysiological alterations predisposed to higher mortality. In several cases early alterations proved to be reversible. ]

Clinical Neuroscience

MAY 25, 2014

[Temperature sensitivity of some nerve conduction parameters in diabetic polyneuropathy]

HIDASI Eszter, DIÓSZEGHY Péter, KÁPLÁR Miklós, MECHLER Ferenc, BERECZKI Dániel

[Background - We tested whether in diabetic polyneuropathy the temperature dependence of the median nerve conduction parameters reflects the severity of neuropathy. Methods - We validated an electrophysiological score against clinical signs of polyneuropathy. Electroneurography was performed at temperatures from 20-40 °C in diabetic patients with mild, moderate and severe neuropathy and controls. Results - The electrophysiological score reflected the clinical severity of polyneuropathy. At room temperature there were significant differences among groups in almost all parameters. In thermal sensitivity studies were significant differences in distal and proximal motor and sensory areas and in sensory conduction velocities. These four parameters normalized to 1 °C change in temperature also significantly differed among the four groups and were largest in controls and smallest in severe polyneuropathy. Conclusions - The use of an integral parameter - areas are essentially amplitudes integrated over time - increases the probability of detecting decreased thermal sensitivity of peripheral nerves in diabetes.]

Clinical Neuroscience

MAY 10, 2005

[A case history and diagnostical rewiev of primary cerebral angiitis]

CSÉPÁNY Tünde, KOLLÁR József, SIKULA Judit, MOLNÁR Mária, CSIBA László

[The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.]

Clinical Neuroscience

OCTOBER 20, 2002

[Kennedy’s syndrome - bulbo-spinal muscular atrophy]

SZABÓ Antal, MECHLER Ferenc

[Kennedy syndrome is a late-onset, bulbar-spinal type of muscular atrophy, with X-linked recessive inheritance. The characteristic features of the disease become prominent in the 4-5th decades: proximal muscle wasting and weakness, bulbar signs, fasciculations in skeletal muscles, subtle signs of endocrine dysfunction, such as gynaecomastia or testicular atrophy. The electrophysiological examinations are the keypoint to the diagnosis. Electroneurography shows normal conduction velocity in peripheral nerves, but the sensory nerves usually show axonal degeneration, which causes only very mild or subclinical neurological deficits. Electromyography shows chronic anterior horn cell degeneration in skeletal muscles. Molecular genetic diagnosis was introduced in 1991, when an abnormal expansion of CAG repeat was found in the first exon of the androgen receptor gene on chromosome X with a frequency of 100% in the affected population. Since the progression is very slow and these patients can expect a normal life span, it is essential to distinguish this syndrome from other, often more severe diseases, such as ALS. There is no proven therapy for Kennedy's disease yet. This is the first case of Kennedy's disease published in Hungary.]