- Email: [email protected]
18. Non-dystrophinopathy muscular dystrophies at childhood age
Society Proceedings / Clinical Neurophysiology 120 (2009) e127–e132
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region, and their response to intravenous benzodiazepines. Following the initiation of antiepileptic treatment, neurological examination normalized concomitantly with resolution of the NCSE. The patient became alert without right hemiparesis. Blood examinations revealed leukocytosis 25.0 with lymphocyte count 17.0 (68.0%). Haematologist diagnosed chronic lymphocytic leukaemia, clinical stage 0 (CLL Rai 0). Conclusions: We recommend considering an early EEG in the evaluation of patients with an acute confusional state. NCSE can have unusual clinical manifestations, and a high index of suspicion is necessary to correctly diagnose these patients.
FSHD in 5 (12.19%) patients. In 32 (78%) patients, EMG diagnostics was carried out. In KMD group of 12 patients in 11 (92%) EMG was myopathic. In 6 out of 7 KMD biopsy was in accordance with EMG findings. In 3 out of 9 MD myotonic discharge was not detected, 1 out of 3 belonged to congenital type MD. EMG in FSHD group was myopathic in 3 out of 4 (75%), in LGMD in 6 out of 7 (5.71%) cases. Conclusions. Sensitivity of EMG test is greatest in KMD group, the least in the MD group. In MD the phenotype specificity and availability of genetic diagnostics transcended low sensitivity of EMG test.
doi:10.1016/j.clinph.2008.12.020
doi:10.1016/j.clinph.2008.12.022
17. West syndrome: Our clinical experience in 36 cases—M. Borkovic´ (Clinic of Neurology and Psychiatry for Children and Youth, Belgrade, Serbia)
19. Anterior interosseous nerve lesion – Case report—Z. Vukojevic´ 1, A. Dominovic´-Kovacˇevic´ 1, S. Grgic´ 1, D. Racˇic´ 1, S. C´ato 2 (1 Banja Luka, Neurological Clinic, Bosnia and Herzegovina, 2 General Hospital Prijedor)
Purpose: To analyze some clinical aspects of patients with West syndrome (WS). Methods: Thirty-six patients were enrolled in retrospective study. Mean onset of epileptic spasms was 6.5 months. All children were followed from one to four years (mean 2.8). Results: Aetiology of epileptic spasms was symptomatic 28 (77.7%). Idiopathic WS was concluded in 8 (22.2%). Family history for epileptic spasms was reported in two girls. Neuroimaging studies showed morphological abnormalities in 77.7%. Steroid therapy was administered in 80.5% of patients, with 75.0% receiving it as initial treatment. Complete or nearly complete control of spasms after one-month treatment was achieved in 25 (75% of patients treated with steroids and in 25.0% receiving vigabatrin). In addition, complete seizure control after one-year follow-up was noted in 11 (30.5%). Significant, favourable decrease in seizure frequency was seen in 26 (72.0%). Normal or slightly delayed psychomotor development was observed in only 9 (25.0%). During the follow-up, (at least one month after the introduction of therapy) focal EEG abnormalities were recorded in 17 (47.2%), modified hypsarrhythmic pattern in 9 (25.0%) and non-specific abnormalities in 10 (27.7%). Conclusion: The majority of our patients have a poor prognosis. Aetiology of WS seemed to have greatest impact on clinical course and long-term outcome.
Introduction: Compression of the anterior interosseous nerve, the biggest branch of the median nerve, in the forearm area is extremely rare in clinical practice (much rarer than carpal tunnel syndrome). It frequently occurs because of compression of the nerve between the two heads of the pronator teres; it may be posttraumatic or idiopathic. Case report: A 45-year-old patient came for the EMG testing due to pains in the right forearm, hand weakness and hyperextension of the index finger. One month before, he had performed an intensive physical work all day long (carrying heavy crates on his forearms). The following days he had felt pains, paresthesia in the front part of the forearm, reduced hand ability, hyperextension of the index finger. The EMG test showed moderately strong signs of the current and chronic neurogenic lesion in the M. Flexor pollicis longus, M. Flexor digitorum profundus and m. pronator quadratus. The patient underwent physical therapy. After three months, subjective complaints have been less expressed, while the EMG finding was improved. Conclusion: Lesions of the anterior interosseous nerve are rare in clinic practice and can cause considerable hand weakness. The EMG is a key diagnostic method, while medical treatment is most often conservative. doi:10.1016/j.clinph.2008.12.023
doi:10.1016/j.clinph.2008.12.021
18. Non-dystrophinopathy muscular dystrophies at childhood age—B. Vukadinovic´-Pajovic´ 1, V. Milic´-Rašic´ 2, S. Todorovic´ 2 (1 Kovin Special Neuropsychiatric Hospital, Kovin, Serbia, 2 Clinic for Neurology and Psychiatry for Children and Youth, Belgrade, Serbia) Introduction: Muscular dystrophies are the most common hereditary myopathies related to children,and include: Dystrophinopathies, Limb-Girdle Muscular Dystrophy (LGMD), Congenital (KMD), Facioscapularohumeral (FSHD), Emery-Dreifuss Muscular Dystrohies, Distal and Myotonic Dystrophies (MD). Purpose: To define frequency of certain non-dystrophinopathy muscular dystrophy entities in childhood, and to identify significance of certain diagnostic procedures, primarily EMG and muscular biopsies. Methods: Registers of hospitalised patients and outpatients from 1997 to 2007 were used. Results: Out of 41 patients, dystrophy diagnosis was differentiated as: KMD 16 (39.02%), MD 12 (29.27%), LGMD 8 (19.51%) and
20. Correlation of EMNG findings in patients taking peroral antidiabetics and insulin—A. Dominovic´-Kovacˇevic´, Z. Vukojevic´, S. Grgic´. D. Racˇic´ (Banja Luka Neurological Clinic, Bosnia and Herzegovina) Diabetic neuropathies represent the most common and the most important complication of diabetes. Goal: To compare EMNG findings in patients taking peroral antidiabetics and insulin. Material and methods: EMNG examination encompassed 48 diabetics (24 taking oral and 24 insulin therapy since the beginning of disease). The average lifetime is 58.9, while the duration of diabetes is 13.9 years. 31 men (64.58%) and 17 women (35.42%) were examined. The following comparisons were made: motor and sensitive conduction velocity, amplitudes of the examined nerves and degree of muscle weakness. Results: The average motor conduction velocity of n. peroneus in patients taking insulin therapy was 40.29 m/s (±4.2), while in patients taking oral therapy 32.83 m/s (±4.7); for N. tibialis it was 40.91 m/s (±2.3) taking insulin, and 34.16 m/s (±2.1) taking oral
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region, and their response to intravenous benzodiazepines. Following the initiation of antiepileptic treatment, neurological examination normalized concomitantly with resolution of the NCSE. The patient became alert without right hemiparesis. Blood examinations revealed leukocytosis 25.0 with lymphocyte count 17.0 (68.0%). Haematologist diagnosed chronic lymphocytic leukaemia, clinical stage 0 (CLL Rai 0). Conclusions: We recommend considering an early EEG in the evaluation of patients with an acute confusional state. NCSE can have unusual clinical manifestations, and a high index of suspicion is necessary to correctly diagnose these patients.
FSHD in 5 (12.19%) patients. In 32 (78%) patients, EMG diagnostics was carried out. In KMD group of 12 patients in 11 (92%) EMG was myopathic. In 6 out of 7 KMD biopsy was in accordance with EMG findings. In 3 out of 9 MD myotonic discharge was not detected, 1 out of 3 belonged to congenital type MD. EMG in FSHD group was myopathic in 3 out of 4 (75%), in LGMD in 6 out of 7 (5.71%) cases. Conclusions. Sensitivity of EMG test is greatest in KMD group, the least in the MD group. In MD the phenotype specificity and availability of genetic diagnostics transcended low sensitivity of EMG test.
doi:10.1016/j.clinph.2008.12.020
doi:10.1016/j.clinph.2008.12.022
17. West syndrome: Our clinical experience in 36 cases—M. Borkovic´ (Clinic of Neurology and Psychiatry for Children and Youth, Belgrade, Serbia)
19. Anterior interosseous nerve lesion – Case report—Z. Vukojevic´ 1, A. Dominovic´-Kovacˇevic´ 1, S. Grgic´ 1, D. Racˇic´ 1, S. C´ato 2 (1 Banja Luka, Neurological Clinic, Bosnia and Herzegovina, 2 General Hospital Prijedor)
Purpose: To analyze some clinical aspects of patients with West syndrome (WS). Methods: Thirty-six patients were enrolled in retrospective study. Mean onset of epileptic spasms was 6.5 months. All children were followed from one to four years (mean 2.8). Results: Aetiology of epileptic spasms was symptomatic 28 (77.7%). Idiopathic WS was concluded in 8 (22.2%). Family history for epileptic spasms was reported in two girls. Neuroimaging studies showed morphological abnormalities in 77.7%. Steroid therapy was administered in 80.5% of patients, with 75.0% receiving it as initial treatment. Complete or nearly complete control of spasms after one-month treatment was achieved in 25 (75% of patients treated with steroids and in 25.0% receiving vigabatrin). In addition, complete seizure control after one-year follow-up was noted in 11 (30.5%). Significant, favourable decrease in seizure frequency was seen in 26 (72.0%). Normal or slightly delayed psychomotor development was observed in only 9 (25.0%). During the follow-up, (at least one month after the introduction of therapy) focal EEG abnormalities were recorded in 17 (47.2%), modified hypsarrhythmic pattern in 9 (25.0%) and non-specific abnormalities in 10 (27.7%). Conclusion: The majority of our patients have a poor prognosis. Aetiology of WS seemed to have greatest impact on clinical course and long-term outcome.
Introduction: Compression of the anterior interosseous nerve, the biggest branch of the median nerve, in the forearm area is extremely rare in clinical practice (much rarer than carpal tunnel syndrome). It frequently occurs because of compression of the nerve between the two heads of the pronator teres; it may be posttraumatic or idiopathic. Case report: A 45-year-old patient came for the EMG testing due to pains in the right forearm, hand weakness and hyperextension of the index finger. One month before, he had performed an intensive physical work all day long (carrying heavy crates on his forearms). The following days he had felt pains, paresthesia in the front part of the forearm, reduced hand ability, hyperextension of the index finger. The EMG test showed moderately strong signs of the current and chronic neurogenic lesion in the M. Flexor pollicis longus, M. Flexor digitorum profundus and m. pronator quadratus. The patient underwent physical therapy. After three months, subjective complaints have been less expressed, while the EMG finding was improved. Conclusion: Lesions of the anterior interosseous nerve are rare in clinic practice and can cause considerable hand weakness. The EMG is a key diagnostic method, while medical treatment is most often conservative. doi:10.1016/j.clinph.2008.12.023
doi:10.1016/j.clinph.2008.12.021
18. Non-dystrophinopathy muscular dystrophies at childhood age—B. Vukadinovic´-Pajovic´ 1, V. Milic´-Rašic´ 2, S. Todorovic´ 2 (1 Kovin Special Neuropsychiatric Hospital, Kovin, Serbia, 2 Clinic for Neurology and Psychiatry for Children and Youth, Belgrade, Serbia) Introduction: Muscular dystrophies are the most common hereditary myopathies related to children,and include: Dystrophinopathies, Limb-Girdle Muscular Dystrophy (LGMD), Congenital (KMD), Facioscapularohumeral (FSHD), Emery-Dreifuss Muscular Dystrohies, Distal and Myotonic Dystrophies (MD). Purpose: To define frequency of certain non-dystrophinopathy muscular dystrophy entities in childhood, and to identify significance of certain diagnostic procedures, primarily EMG and muscular biopsies. Methods: Registers of hospitalised patients and outpatients from 1997 to 2007 were used. Results: Out of 41 patients, dystrophy diagnosis was differentiated as: KMD 16 (39.02%), MD 12 (29.27%), LGMD 8 (19.51%) and
20. Correlation of EMNG findings in patients taking peroral antidiabetics and insulin—A. Dominovic´-Kovacˇevic´, Z. Vukojevic´, S. Grgic´. D. Racˇic´ (Banja Luka Neurological Clinic, Bosnia and Herzegovina) Diabetic neuropathies represent the most common and the most important complication of diabetes. Goal: To compare EMNG findings in patients taking peroral antidiabetics and insulin. Material and methods: EMNG examination encompassed 48 diabetics (24 taking oral and 24 insulin therapy since the beginning of disease). The average lifetime is 58.9, while the duration of diabetes is 13.9 years. 31 men (64.58%) and 17 women (35.42%) were examined. The following comparisons were made: motor and sensitive conduction velocity, amplitudes of the examined nerves and degree of muscle weakness. Results: The average motor conduction velocity of n. peroneus in patients taking insulin therapy was 40.29 m/s (±4.2), while in patients taking oral therapy 32.83 m/s (±4.7); for N. tibialis it was 40.91 m/s (±2.3) taking insulin, and 34.16 m/s (±2.1) taking oral