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Clinical Neuroscience

NOVEMBER 30, 2017

Nerve conduction study and gastrocnemius H reflex response in rheumatoid arthritis

EMRE Ufuk, ORTANCIL Özgül, UNAL Aysun, KIRAN Sibel, SAPMAZ Perihan, ATASOY Tugrul

Objectives - We aimed to evaluate nerve conduction studies and gastrocnemius H reflex responses in rheumatoid arthritis (RA) patients and compared to the healthy adult subjects. Materials and methods - Twenty-six RA patients and twenty-two healthy adult subjects were included in the study. The nerve conduction study (NCS) findings and bilateral gastrocnemius H reflex responses were evaluated in all the groups. Age, gender, subcutaneous nodules, joint deformities, laboratory parameters, duration of disease, anti-rheumatic drug and steroid usage were recorded. Activity of disease was assessed using a 28-joint disease activity score (DAS28).The functional status was measured using the health assessment questionnaire (HAQ), pain intensity measured using a visual analog scale (VAS). Results - The rate of electroneuromyographic (ENMG) abnormalities was 73% in RA patients. The most common diagnosis was carpal tunnel syndrome (61.4%). There were no significant correlations between ENMG findings and clinical and laboratory features evaluated. Right H reflex latencies were statistically longer in RA patients (p=0.03). According to calculated cut-off levels, there were more subjects with longer H reflex latencies in RA patients. Conclusions - In this study, entrapment neuropathies were found common as independent identity from duration and severity of disease in RA patients. For H reflex latencies, cut-off values were longer in RA patients. It may provide information about the early neuropathic involvement of long peripheral nerves in RA patients. But this findings are needed to be supported by larger population study.

Clinical Neuroscience

SEPTEMBER 30, 2017

Correlation of vitamin D levels with electrophysiological findings and pain in the patients with Carpal Tunnel Syndrome

DEMIRYÜREK Enes Bekir, SENTÜRK Asli

Objective - This study aimed to assess the correlation between vitamin D deficiency and electrophysiological findings and pain level in patients with symptoms of carpal tunnel syndrome (CTS). Patients and method - A total of 131 patients with symptoms of CTS, 70 with vitamin D deficiency and 61 without vitamin D deficiency, were included in the study. Using demographic data and findings from electrophysiological examinations, the patients were divided into two groups based on their vitamin D level (Group 1: <20 ng/ml; Group 2: ≥20 ng/ml). The Boston Carpal Tunnel Syndrome Questionnaire was used to assess their CTS- related pain level. Results - Although the rate of CTS in the patients with a low vitamin D level was found to be high, no statistically significant correlation was observed between low vitamin D level and the frequency and severity of CTS. Additionally, the pain and functional loss ratio induced by CTS was found to be higher in the group with a lower vitamin D level than in the group with normal levels. Conclusion - Low vitamin D levels may increase the severity of CTS symptoms. Treatment of vitamin D deficiency in patients with CTS can play a role in reducing pain and disability.

Clinical Oncology

SEPTEMBER 15, 2016

[Cancer-treatment induced peripheral neuropathy]

DEMETER Gyula

[Peripheral neuropathy is caused by structural or functional damage of nervous system. The pathophysiology is not well known. Its clinical features are established but there is a need to standardize CIPN assessment, also considering that health care providers and patients frequently have a different perception of CIPN severity. Neurotoxicity caused by traditional chemotherapy is widely recognized in patients with cancer. The adverse effects of newer therapeutics, such as targeting and immunotherapeutic agents, need more information for the proper management. This review addresses the main neurotoxicities of cancer treatments with a focus on the newer therapeutics. Recognition of these patterns of toxicity is important because drug discontinuation or dose adjustment might prevent further neurological injury. Treatment is symptomatic. For prevention or treatment there is need for further basic research outcomes.]

Clinical Neuroscience

JULY 30, 2016

[Transthyretin familial amyloid polyneuropathy - three Hungarian cases with rare mutations (His88Arg and Phe33Leu)]

CSILLIK Anita, POZSONYI Zoltán, SOÓS Krisztina, BALOGH István, BODÓ Imre, ARÁNYI Zsuzsanna

[Introduction - Transthyretin familial amyloid polyneuropathy is a rare autosomal dominant progressive systemic disesase of adults caused by endoneural amyloid deposition due to point mutations of the transthyretin gene. It is the most severe form among hereditary polyneuropathies, being fatal within 10 years if left untreated. The disease is underdiagnosed, the late onset forms (above the age of 50) being probably more widespread than previously thought. Early diagnosis is essential as the early introduction of causal therapy (tafamidis) slows progression and prolongs survival. Patients - We report here three non-related Hungarian cases of transthyretin familial amyloid polyneuropathy with non- Val30Met mutations (His88Arg in two cases, Phe33Leu in one case). They were all characterized by late-onset, progressive, length-dependent, axonal, sensorimotor polyneuropathy and the simultaneous presentation of severe restrictive cardiomyopathy. In all three cases, clinical and electrophysiological signs of myopathy were also present, suggesting the involvement of skeletal muscles as well. In two cases, high resolution ultrasound of the peripheral nerves was also performed, which showed segmental structural alterations (change or loss of fascicular structure) and some increase of echogenicity of the interfascicular epineurium, without substantial enlargement of the nerves. Conclusion - In Hungary, mainly the rare, non-Val30Met mutation forms of transthyretin familial amyloid polyneuropathy are encountered, as in our cases. As opposed to the Val30Met forms, these mutations are characterized by late onset and simultaneous presentation of severe cardiomyopathy. Our report highlights the importance of considering transthyretin familial amyloid polyneuropathy in the differential diagnosis of late-onset, progressive, axonal polyneuropathies of unknown etiology, particularly if associated with cardiac disease.]

Clinical Neuroscience

SEPTEMBER 30, 2015

Quality of life of patients with non-diabetic peripheral neuropathic pain; results from a cross-sectional survey in general practices in Hungary

BRODSZKY Valentin, PÉNTEK Márta, KOMOLY Sámuel, BERECZKI Dániel, EMBEY-ISZTIN Dezső, TORZSA Péter, GULÁCSI László

Background and purpose - There is a lack of data on the impact on health related quality of life of peripheral neuropathic pain in Hungary. The main aims of the study were to assess the health related quality of life of nondiabetic PeNP patients identified in general practices through screening, and to assess the relationship between condition specific pain scores and health state utilities. Methods - Non-diabetic patients aged ≥30 years were recruited in 10 general practices in Hungary. At first, patients filled in the PainDETECT Questionnaire (PD-Q) and those who have achieved ≥13 PD-Q score (unclear or possible neuropathic pain) were further assessed by the DN4 questionnaire. Patients with PD-Q score >18 or DN4 score ≥4 were considered to have PeNP and they completed the EQ-5D health questionnaire. Results - Among the 111 patients identified as non-diabetic PeNP patients the mean age was 62 (SD=14) years, 69% were women. Average EQ-5D score was 44% lower than the gender and age matched Hungarian norm (0.42 vs. 0.75, p<0.001) and it worsened with increasing pain intensity. The pain/discomfort and anxiety/depression were the most affected EQ-5D dimensions. Strong relationship was demonstrated between the PD-Q and EQ- 5D score. Most of the PeNP patients (86%) were undiagnosed. Conclusions - Non-diabetic PeNP pain has a huge negative impact on health related quality of life. Although PeNP is a serious chronic condition, the disease burden is seriously underestimated, both on the level of individuals and society, due to the fact that patients are rarely identified.

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. ]

LAM Extra for General Practicioners

DECEMBER 05, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]

Clinical Neuroscience

NOVEMBER 28, 2014

[Critical illness associated neuromuscular disorders - Keep them in mind]

NEMES Réka, MOLNÁR Levente, FÜLEP Zoltán, FEKETE Klára, BERHÉS Mariann, FÜLESDI Béla

[Neuromuscular disorders complicating sepsis and critical illness are not new and scarce phenomena yet they receive little attention in daily clinical practice. Critical illness polyneuropathy and myopathy affect nearly half of the patients with sepsis. The difficult weaning from the ventilator, the prolonged intensive care unit and hospital stay, the larger complication and mortality rate these disorders predispose to, put a large burden on the patient and the health care system. The aim of this review is to give an insight into the pathophysiological background, diagnostic possibilities and potential preventive and therapeutic measures in connection with these disorders to draw attention to their significance and underline the importance of preventive approach.]

Clinical Neuroscience

NOVEMBER 28, 2014

[The modifying effect a PMP22 deletion in a family with Charcot-Marie-Tooth type 1 neuropathy due to an EGR2 mutation]

REMÉNYI Viktória, INCZÉDY-FARKAS Gabriella, GÁL Anikó, BEREZNAI Benjámin, PÁL Zsuzsanna, KARCAGI Veronika, MECHLER Ferenc, MOLNÁR Mária Judit

[Background - Mutations of both the PMP22 and EGR2 genes cause Charcot-Marie-Tooth (CMT) disease type 1. Deletion of the PMP22 gene, results in hereditary neuropathy with liability to pressure palsies. More publications exist about the interaction of PMP22 duplication and other CMTcausing gene mutations. In these cases the intrafamiliar discordant phenotypes draw the attention to the possible role of modifying genes. The gene-gene interactions between the PMP22 and EGR2 genes are not well understood. Case report - We report two brothers with late onset CMT1 due to a c. 1142 G>A (Arg381His) heterozygous substitution in the EGR2 gene. Additionally, the older brother with the less severe symptoms harbored the PMP22 gene deletion also. Conclusion - The coexistence of the two genetic alterations did not aggravate the clinical symptoms. Moreover, the PMP22 deletion appeared to have a beneficial modifying effect, thus implying potential gene-gene interaction of PMP22 and EGR2. PMP22 deletion may increase Schwann cells proliferation and compensate the dominant-negative effect of the Arg381His substitution in the EGR2 gene.]

Lege Artis Medicinae

SEPTEMBER 21, 2014

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]