Marta Waliszewska-Prosół

Endogenous event-related potentials in patients with primary Sjögren’s syndrome without central nervous system involvement

Objectives: Endogenous cognitive event-related potentials (CERPs) reflect higher-level processing of sensory information and can be used to evaluate cognitive functions. The aim of this paper was to determine whether there are any abnormalities in the electrophysiological parameters of CERPs in patients with primary Sjögren’s syndrome (pSS) but without symptoms of central nervous system (CNS) involvement or mental disorder. The analysis of CERP parameters was then correlated with the clinical status of the patients and with some of the immunological parameters in the patient group. Method: Thirty consecutive patients with pSS (29 females, one male) were included in the study. All the patients underwent CERP examination. Results: There was a significant prolongation of the latency of P300 and N200 potentials in patients with pSS. Abnormalities in electrophysiological parameters of CERPs correlated with the duration of the disease, salivary gland abnormalities, and elevated erythrocyte sedimentation rate (ESR) values. Patients with coexisting chronic fatigue syndrome (CFS) had larger P300 amplitudes. There were no statistically significant changes in the electrophysiological parameters of CERPs in patients with pSS dependent on the presence of peripheral nervous system (PNS) lesions, skin changes, arthritis, abnormalities in white blood cells and the immune system or the levels of blood lipids. Conclusions: The results of the study suggest the presence of a minor cognitive dysfunction in patients with pSS without symptoms of CNS involvement or mental disorder. Cognitive dysfunction correlated with the disease duration time and the severity of inflammatory changes (salivary gland abnormalities and inflammatory markers in the blood). Further and larger longitudinal studies are necessary for confirmation of this correlation.

Cerebellar ataxia as a first manifestation of Creutzfeldt-Jakob disease in two cousins

A 56-year-old man without a significant past medical history was admitted to the Neurology Department because of an 8-month history of progressive cerebellar syndrome and a 4-month history of progressing dementia. The neurological examination …

Severe course of neuromyelitis optica in a female patient with chronic C hepatitis

Neuromyelitis optica (NMO) is a rare, disabling, recurring inflammatory demyelinating disease affecting the spinal cord and optic nerves with predominance in women. We present the case of a female patient with chronic C hepatitis, who, despite treatment, developed severe symptoms of NMO during pregnancy and postpartum.

BS14. Visual and brainstem auditory evoked potentials in Hashimoto’s thyroiditis

Introduction A normal thyroid gland activity is essential for the optimal development, maturation and function of the nervous system. Neurological syndromes can develop in certain thyroid diseases that may have normal levels of thyroid hormones. One such example is Hashimoto’s thyroiditis (HT), which is currently considered to be the most common autoimmune disease in humans. The exact cause of those symptoms has not yet fully been explained. Their pathomechanism is still being studied. Precise and non-invasive methods of assessment of the nervous system function is evoked potentials study (EP). It is a sensitive technique that enables the evaluation of the brain bioelectrical activity also in patients with HT. EPs are particularly useful in patients with mild or without clinical neurological symptoms. The aim of the study was to evaluate parameters of visual and brainstem auditory evoked potentials (VEP, BAEP) in euthyreotic HT patients without clinical neurological deficit, and without ophthalmological and otolaryngological abnormalities. Methods 100 HT patients (92 women, 8 men) from 20 to 68 years old (mean 46.9 years). They did not suffer from concurrent diseases that could have affected the brain electrical activity. The visual and auditory receptors were not damaged in any of the patients and the patients did not suffer from any central nervous system damage. The matched control group comprised 50 healthy subjects. HT patients underwent a subjective and objective neurological examination, thyroid hormone levels and thyroid autoantibody titers, brain imaging (CT or MRI), VEP and BAEP. Results Abnormal VEP and BAEP were recorded in 34% of the patients. The mean P100, relative P100, N145 VEP latencies were significantly longer and P100 amplitude significantly higher in HT patients than in the control group. HT patients also had a longer mean wave V latency and mean wave III-V and I-V interpeak latencies and significantly lower mean wave I and V amplitudes than the controls. There were no statistically significant correlations between the mean VEP parameters and the levels of TSH, free thyroid hormones, antithyroid antibody titers and the applied dose of L-thyroxine. There was a relationship between the level of TSH and the wave BAEP III-V interpeak latency. The number of abnormal VEP and BAEP depended on the dose of L-thyroxine and was the highest in patients without treatment and decreased with the increase in the dose of levothyroxine. Conclusion 1. There were changes in the brain bioelectrical activity in the form of abnormal VEP and BAEP in one third of the patients with Hashimoto’s disease who did not have central nervous system deficits. 2. The increased amplitude of the VEP in the course of HT may indicate increased cerebral cortex activity. 3. Disorders of the brain bioelectrical activity in the course of HT may be associated with an ongoing autoimmune process. 4. The analysis of the VEP and BAEP may be useful in assessing the brain bioelectrical activity in the course of HT.

Parameters of Somatosensory Evoked Potentials in Patients with Primary Sjӧgren’s Syndrome: Preliminary Results

Primary Sjogrens syndrome (pSS) is a chronic autoimmune disease. The aim of the study was to establish whether in patients with pSS without central nervous system (CNS) involvement, the function of the central portion of the sensory pathway can be challenged. In 33 patients with pSS without clinical features of CNS damage and normal head computed tomography scan, somatosensory evoked potentials (SEP) were studied. The results were compared to other clinical parameters of the disease, particularly to immunological status. The control group consisted of 20 healthy volunteers. Mean latency of all components of SEP was considerably prolonged in patients compared to the control group. Mean interpeak latency N20-N13 (duration of central conduction TT) did not differ significantly between the groups. However, in the study group, mean amplitude of N20P22 and N13P16 was significantly higher compared to healthy individuals. In patients with pSS, significant differences in SEP parameters depending on the duration of the disease and presence of SSA and SSB antibodies were noted. The authors confirmed CNS involvement often observed in patients with pSS. They also showed dysfunction of the central sensory neuron as a difference in the amplitude of cortical response, which indicates subclinical damage to the CNS.

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids Syndrome: Diagnostic Challenge of the Brainstem Inflammation

resis of left limbs with pyramidal signs and exaggerated reflexes. Brain MRI showed changes typical of the chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids ( Fig. 1 ) [1] . Laboratory blood A 57-year-old man was admitted to the Neurology Department because of the 9-month history of progressive cerebellar-pyramidal syndrome with accompanied headaches. The neurological examination revealed dysarthria, dysphagia, nystagmus, gait ataxia, and the paReceived: November 14, 2016 Accepted: December 6, 2016 Published online: December 21, 2016

Progressive subacute Miller-Fisher syndrome successfully treated with plasmapheresis

BACKGROUND Miller-Fisher Syndrome (MFS) is a rare acute polyneuropathy composed of the clinical triad of ataxia, areflexia and ophthalmoplegia, with a monophasic, self-limited course and spontaneous improvement. CASE REPORT The authors present a 65-year-old man with Miller-Fisher syndrome consisting of bilateral ophthalmoplegia, trigeminal and facial nerve palsy, mild ataxia and peripheral neuropathy. The disease had a progressive, subacute course within 3 months. A high titer of anti-GQ1b antibodies was detected. As a result of plasmapheresis, complete recovery was achieved. CONCLUSIONS The presented case was atypical in its clinical course and treatment. It could support the theory of the continuity between MFS, Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome (GBS).

Rare clinical form of glioblastoma multiforme

Glioblastoma multiforme (glioblastoma multiforme - GBM) is the most malignant tumor classified by WHO. It is also the most common primary CNS tumor with a very aggressive course and unfavourable prognosis, usually develops in adults, and is typically located supratentorially in the fronto-temporal region. However, the literature describes an unusual position of GBM (e.g. spinal cord, pons, pineal region), familial gliomas unconnected with the family of gliomas predisposed to the occurrence of syndromes, unusual glioma and metastatic sites, gliomas transplanted with organs. In this paper, based on the available literature, the authors discuss an unusual and rare form of glioblastoma multiforme.

P117: Quantitative EEG alterations in alcohol-dependent patients with epileptic seizures – pilot study

Main parameters evaluated in the study were age at onset of epilepsy, seizure characteristics as type, frequency, duration, relation with sleepwake cycle, patient and family history for paroxysmal disorders and clinical course of the seizures. Distribution of interictal spike-wave in the EEGs were analyzed and their localizations were questioned in relation to age of the patient at the time of recording. Number of patients with typical BRE was 17, PS 9, Childhood Epilepsy with Occipital Paroxsyms (Gastaut-type) 8 and ABPE 1. Remaining patients exhibited either characteristics common for more than one syndrome, or, some features within the borderlines of IPEs. Ratio of patients with idiopathic epilepsy in at least one family member was % 38 and with parental consanguinity was % 8 Spike distribution in the EEGs was not only related to the syndromic characteristics of the patients but also to the age at the EEG recording. Results are discussed in view of the related literature.

An Older Woman after a First Generalized Seizure

An 81-year-old woman without a significant past medical history was admitted to our Neurol- ogy Department following a first generalized seizure. Her physical exam was normal except for confusion and psychomotor slowing. Head CT was normal. Laboratory tests on serum and CSF (including tests for HIV and other infectious diseases) were unremarkable. Conventional brain MR examination revealed multiple enhancing lesions, which suggested a metastatic brain disease ( fig. 1 ). Many diagnostic procedures were performed (chest CT, abdomen CT, tumor markers, immunophenotype of lymphocytes in CSF), and these allowed the exclusion of primary neoplas-matic processes. Our patient did not agree to a bone marrow biopsy; however, she did consent to a brain biopsy.