Long-standing myoclonic hand tremor as an isolated symptom of hypertrophic olivary degeneration.

Hypertrophic olivary degeneration (HOD) is a rare condition caused by lesions of the dentato-rubro-olivary pathway, usually bilateral. We presented a case of a 64-year old male with HOD caused by a unilateral, posterior pontine cavernoma. The patient has not developed the typical palate myoclonus until recently. Isolated hand myoclonus with coexisting asterixis was present for years. This case shows unique HOD symptomatology and emphasizes the important role of MRI in the differential diagnosis of monomelic myoclonus.

The Relevance of Collision Tests and Quantitative Sensory Testing in Diagnostics and Postoperative Outcome Prediction in Carpal Tunnel Syndrome.

Background: The gold standards for the diagnosis and treatment of carpal tunnel syndrome (CTS) and its outcome are undecided. Using clinical and electrophysiological methods, we tried to establish which fibers achieved full postoperative recovery, and the possibility of using non-standard electrophysiological tests as outcome predictors. Methods: The study group consisted of 35 patients and controls. The Historical-Objective Scale, standard neurography, conduction velocity distribution tests (CVD), and quantitative sensory testing (QST) were performed before and after CTS surgery. Results: Clinical improvement was observed on average in 54.3% of the patients, higher in less advanced CTS. All parameters improved significantly after surgery, except for CVD; most remained worse than in the controls. Only QST parameters fully returned to normal limits. Patient age and CTS severity were important in the estimation of the risk of no improvement. Conclusions: The efficiency of minimally invasive CTS surgery is higher in younger patients with less advanced CTS. Complete recovery was present only in small fibers; larger fibers could most likely be responsible for residual signs. We did not notice any benefits in CTS diagnosis using methods of small fiber assessment. QST seemed to be useful in the diagnosis of residual signs, and in deciding upon possible reoperation.

The Role of Stem Cells in the Therapy of Stroke.

BACKGROUND: Stroke is a major challenge in neurology due to its multifactorial genesis and irreversible consequences. Processes of endogenous post-stroke neurogenesis, although insufficient, may indicate possible direction of future therapy. Multiple research considers stem-cell-based approaches in order to maximize neuroregeneration and minimize post-stroke deficits. OBJECTIVE: Aim of this study is to review current literature considering post-stroke stem-cell- based therapy and possibilities of inducing neuroregeneration after brain vascular damage. METHODS: Papers included in this article were obtained from PubMed and MEDLINE databases. The following medical subject headings (MeSH) were used: "stem cell therapy", "post-stroke neurogenesis", "stem-cells stroke", "stroke neurogenesis", "stroke stem cells", "stroke", "cell therapy", "neuroregeneration", "neurogenesis", "stem-cell human", "cell therapy in human". Ultimate inclusion was made after manual review of the obtained reference list. RESULTS: Attempts of stimulating neuroregeneration after stroke found in current literature include supporting endogenous neurogenesis, different routes of exogenous stem cells supplying and extracellular vesicles used as a method of particle transport. CONCLUSION: Although further research in this field is required, post stroke brain recovery supported by exogenous stem cells seems to be promising future therapy revolutionizing modern neurology.

The Relationship between Occupationally Exposed Arsenic, Cadmium and Lead and Brain Bioelectrical Activity-A Visual and Brainstem Auditory Evoked Potentials Study.

The aim of this study was to evaluate the parameters of visual and brainstem auditory evoked potentials in patients occupationally exposed to arsenic, cadmium and lead. The study group comprised 41 copper smelter and refinery workers (average age: 51.27) with occupational exposure to arsenic, cadmium and lead. The control group consisted of 36 healthy volunteers (35 men and 1 woman, aged 27-66, average age: 51.08). Neurological examination, brain imaging, and visual and brainstem auditory evoked potentials were performed, and the relationship between blood Cd, Pb concentration (Cd-B, Pb-B), blood zinc protoporphyrin (ZnPP), and urine As concentration (As-U) were assessed. In the workers, exceedances of allowable biological concentrations were observed, with the urinary concentration of arsenic being 5.2%, the cadmium and lead in blood being 1.3%, while the case of ZnPP was 2.6%. The mean P100, relative P100, and N145 visual evoked potential (VEP) latencies were significantly longer in exposed workers than in the controls. The mean wave III and V brainstem auditory evoked potential (BAEP) latency and the mean wave III-V and I-V interpeak latencies were longer, and the I and V amplitude was lower in the workers than the controls. In summary, occupational exposure to As, Cd, and Pb is associated with prolonged latency and reduced evoked potential amplitude, but As-U, Pb-B, Cd-B, and ZnPP concentrations are not linearly related to potential components. The analysis of evoked potentials may be a useful method of assessment of the central nervous system in patients with occupational exposure to heavy metals.

Opsoclonus-myoclonus syndrome with severe clinical course and beneficial outcome: A case report.

RATIONALE: Opsoclonus-myoclonus syndrome (OMS) is a rare immune-mediated movement disorder, mostly of paraneoplastic or idiopathic origin. The disease usually has an acute onset, serious course and leads rapidly to disability in adult patients. To the best of our knowledge, this is the fourth presented case of OMS with a severe course and complete reversibility of neurological symptoms in a pregnant woman. This report includes videos and a literature review. PATIENT CONCERNS: A 30-year-old woman in the 12th week of pregnancy developed severe nausea and vomiting, after several days balance and gait disorders appeared. On admission to hospital, neurological examination revealed opsoclonus, dysarthria, myoclonic jerks with ataxia of the trunk and limbs with inability to sit, stand or walk. DIAGNOSIS: Well-known causes of OMS were excluded. Although in our patient the idiopathic origin of the disorder was taken under consideration, diagnosis of opsoclonus-myoclonus related to the pregnancy was highly likely. INTERVENTIONS: After administration of steroids and benzodiazepines the patient improved. OUTCOMES: In the 6th month of pregnancy, after termination of immunotherapy, she recovered completely and was able to sit, stand and walk independently. In the 39th week of pregnancy, she delivered a healthy child. LESSONS: We confirm that understanding of clinical symptoms and rare causes of OMS contributes to early diagnosis and therapy, which ensures an optimal outcome. One probable cause of OMS could be a physiological change to immune system regulation during pregnancy. The relationship between OMS and pregnancy remains uncertain and needs further investigation.

Diagnostic Difficulties in Primary Pauci-Melanotic Leptomeningeal Melanomatosis.

We present a rare case of primary malignant melanoma of the central nervous system. We underline the difficulties we faced during diagnostic procedures. Finally, postmortem examination revealed the diagnosis of primary pauci--melanotic leptomeningeal melanomatosis.

Profile of autonomic dysfunctions in patients with primary brain tumor and possible autoimmunity.

OBJECTIVE: Cerebral lesion due to different neurological conditions could be complicated by autonomic dysfunction, reported in the literature as a sympathetic hyperactivity. The mechanisms of dysautonomia still remains partial. The aim of the study was to assess the profile of autonomic dysfunction in patient with primary brain tumors, with attempt to estimate the additional factors in pathogenesis of dysautonomia. MATERIAL AND METHODS: Neurological examinations, the Low's autonomic disorder questionnaire, electrophysiological autonomic tests (Heart Rate Variability test at rest and during deep breathing, spectral analysis of R-R intervals, sympathetic skin response test), studies of peripheral nerves, blood sampling collection for antibodies were done in 33 patients with recognized primary brain tumors. RESULTS: The averaged Low's Questionnaire score in the patients group was significantly higher than in the controls, systolic blood pressure was increased, heart rate tended to be higher without significance, but heart rate variability was severe low, LF/HF ratio also tended to be higher in the patients group. In SSR test the amplitude of responses from hand and foot was significantly lower without changes in their latencies. We found changes in the electrophysiological tests of peripheral nerves, and positive anti-neural antibodies in 5 patients. CONCLUSIONS: The results of the study indicated to the sympathetic nervous system hyperactivity in patients with primary brain tumors. Local brain lesion with high intracranial pressure, additional peripheral nerve damage probably in the course of autoimmunity, and direct influence of autoimmunity to the central part of autonomic nervous system are possible in the pathogenesis of dysautonomia.

Is peripheral paraneoplastic neurological syndrome possible in primary brain tumors?

INTRODUCTION: Systemic malignant diseases cause the induction of autoimmunity, for example, paraneoplastic syndromes. There are no proofs of paraneoplastic syndromes in primary brain tumors. The aim of the study was to evaluate the involvement of the peripheral nervous system, together with an assessment of onconeuronal and antineural antibodies as indicators of humoral immune response against nervous system in patients with primary brain tumors. MATERIALS AND METHODS: Clinical examinations, electrophysiological studies of peripheral nerves (motor and sensory conduction velocity studies, conduction velocity distribution tests, thermal and vibratory quantitative sensory tests, and sympathetic skin response tests) and muscles, blood sampling collection (assessment of onconeuronal, and antineural antibodies) were performed on 33 patients with newly recognized primary brain tumors within 2-4 days after their admission to our department. RESULTS: We revealed statistically significant changes of peripheral nerves, more pronounced in the peroneal nerve in standard and conduction velocity distribution tests, as well as in sympathetic skin responses. We revealed significantly higher vibratory thresholds, and pain thresholds for cold and warm in the upper and lower limbs in the study group than in the controls. In five patients, we have identified anti-neuroendothelium, anti-GFAP, anti-MAG, anti-PCNA, and anti-Ro52 antibodies. CONCLUSIONS: In patients with primary brain tumors, electrophysiological changes in peripheral nerves, together with the presence of the antineural antibodies suggest an autoimmune humoral response, and make the diagnosis of paraneoplastic neurological syndrome possible.

Peripheral nerve tumours: 30-year experience in the surgical treatment.

Peripheral nerve tumours are relatively rare type of soft tissue tumours. The aim of this work is to present our experience with surgical treatment of this type of lesions. Clinical material consists of 94 patients (56 females, 38 males), in whom 101 tumours deriving from peripheral nervous system were removed. The patients underwent surgical treatment between 1983 and 2012. Tumours occurred mainly in the upper extremity (72 tumours), less often in the lower extremity (25 tumours). Lesions developed in major peripheral nerves (51 tumours) and small nerve branches (50 tumours). The most common symptoms reported before surgery included presence of tumour mass (100 %), positive Hoffmann-Tinel sign (95.6 %) and paraesthesia (93.4 %). Less often sensory deficit (89.1 %) and pain (71.7 %) were observed. Motor deficit was the least common manifestation (41.3 %). Benign tumours prevailed in presented material (94 tumours). In 7 cases, malignant peripheral nerve sheath tumour (MPNST) was identified. As a result of surgical treatment in the group of tumours deriving from major peripheral nerves, in 87.8 % of the patients, pain relief was achieved; in 84 %, Hoffmann-Tinel sign was negative; and in 79 %, paraesthesia resolved. Sensory function improvement was observed in 51.2 % of the patients while motor function improved in 26.3 % of the patients. None of the patients experienced tumour relapse. In the group of tumours deriving from small nerve branches, 47 patients had no signs of tumour recurrence. One female patient diagnosed with MPNST suffered a relapse. Obtaining satisfactory results of peripheral nerve tumour treatment requires both careful differential diagnosis and well thought-out strategy at every stage of therapeutic management.

Anatomical variation of the vertebral artery clinically mimicking myasthenia gravis.

Cranial nerve palsy, most commonly trigeminal, abducens, or facial, caused by compression of an ectatic or elongated intracranial artery is a well-known phenomenon. Symptoms of brain stem compression by an abnormal artery have rarely been reported (Tomasello et al. Neurosurgery 56(suppl 1):117-124, 2005). The authors present a 59-year-old woman with intermittent ptosis of the right eye, diplopia and swallowing disturbances, enhanced after physical effort, implying myasthenia gravis. Typical diagnostic procedures, e.g. repetitive nerve stimulation tests, acetylcholine receptor antibodies level were within normal limit. Neurogenic changes from the orbicularis oculi muscle were found in EMG. MRI and angio-CT revealed anatomical variation of the vertebral artery (elongated and arcuate route), causing intermittent signs of brain stem lesion. We point out the similarity of the clinical symptoms of myasthenia gravis and vascular brain stem compression by abnormal vertebral artery. The two diseases require completely different therapeutic proceedings.

[CD40L expression on T CD4+ lymphocytes from peripheral blood in patients with relapsing-remitting and secondary progressive multiple sclerosis]. / Ekspresja receptora CD40L na limfocytach T CD4+ krwi obwodowej u chorych ze zwalniajaca i wtórnie postepujaca postacia stwardnienia rozsianego.

UNLABELLED: Multiple sclerosis (MS) is believed to be a T cell-mediated autoimmune disease. One of the particularly important signals is mediated by CD40L, a costimulatory molecule which appears on activated T cell. The aim of this study was examination of the CD40L expression on freshly obtained lymphocytes T CD4+ which were ex vivo stimulated with monoclonal antibody anti CD3+rIL-2 in patients with relapsing-remitting and secondary progressive multiple sclerosis. MATERIAL AND METHODS: 12 relapsing-remitting (RR) and 16 secondary progressive (SP) MS patients with long-lasting clinical remission and 24 healthy subjects were included in the study. The proportion of unstimulated and ex vivo stimulated with anti CD3+rIL-2 TCD4+ cells from peripheral blood co-expressing CD40L was studied by dual immunofluorescence method. RESULTS: The proportion of unstimulated and stimulated T CD4+CD40L+ cells did not differ significantly between RRMS and controls. The percentage of unstimulated TCD4+CD40L+ cells from SP patients exhibited significantly higher proportion - when compared with controls. These cells did not respond to ex vivo stimulation and their level was similar to that of stimulated cells from controls. CONCLUSION: Dysregulation of costimulation in SPMS expressing as enhanced percentage of TCD4+CD40L+ cells may be responsible for maintenance of chronic activation state of lymphocytes leading to prolonged inflammatory process.