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No. 39 Electromyographic Alterations in Paraspinal Muscles as Unique Finding in a Patient With Deficiency of Alpha Glucosidase
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be highly sensitive and specific in the same way should reduce the number of tests that are used to support a diagnosis of CTS. Methods: 89 patients with a diagnosis of CTS were evaluated. Antidromic median palmar sensory latency (APMSL) was recorded in the palm using an electrode over the motor point of the second lumbrical interossei muscle. Median nerve electrical stimulation was performed using a fixed distance of 8 cm proximal to the active recording electrode at the wrist and the peak latency was used. The low filter was 10 Hz, high filter 2 kHz, sweep duration 2 ms, and sensitivity 20 mv. The abnormal cutoff based upon 168 normal controls was: 2.1 ms (range 1.4-2.1 mean 1.8 SD 0.1). Patients with severe conductions delays were not included. Routine motor and sensory distal latency tests of the median and ulnar nerves were also made. Results: Sensitivity and specificity were 81% and 96%; positive and negative predictive values were 91% and 90%. Summary/ conclusion: To our knowledge there is not a previous description of the proposed new technique. The accuracy of this test in confirming cases of CTS was good. It is a fast, easy to perform test in the diagnosis of CTS and consistently found to be highly sensitive and specific.
No. 39 Electromyographic Alterations in Paraspinal Muscles as Unique Finding in a Patient With Deficiency of Alpha Glucosidase. Pedro A. Escobar, MD; Alvaro E. Rodriguez, MD; Edicson Ruiz; Fernando Ortiz. Disclosure: None. Setting: Orthopedic rehabilitation center in Bogota, Colombia. Patient: Male, 57 years old. Case Description: 7 years evolution of gait difficulties principally in sloping ground but also in climbing stairs and to achieve bipedestation from sitting position. He denied respiratory symptoms, dysphagia or sphincter incontinence. Physical examination showed weakness at shoulder and pelvic girdles. Stretch reflexes were normal and symmetrical. Assessment/Results: Electromyography (EMG) of lower thoracic (below T8 level) and lumbar paraspinal muscles showed membrane instability signs, complex repetitive discharges, fibrillation potentials, and positive sharp waves. Quantitative EMG by interference pattern analysis and motor unit action potentials (MUAP) were normal in quadriceps, gluteus medius and gluteus maximus muscles. Creatine phosphokinase (CPK) 427. Magnetic resonance images (MRI) of brain showed white matter microangiopathy. Lumbar MRI showed atrophy of paraspinal muscles. Acid alpha-glucosidase (GAA) levels: 0.3 mmol/l/h. (normal values > 39 umol/l/h) and double mutation in GAA gene (c.-32-13t>g y c.887del cp.p296lfs*18). Discussion: Disease by deficiency of GAA enzyme produces progressive muscle disorders which leads to abnormal buildup of intracellular glycogen principally in skeletal cardiac and smooth muscles. It leads to weakness, organic failure, and consequently death. Weakness severity is variable because of degree of enzymatic deficiency displaying a wide spectrum of clinical presentation. EMG occasionally shows alterations in paraspinal muscles only. Conclusion: Electrophysiological laboratories in case of suspicion of GAA enzyme deficiency should do paraspinal muscles EMG studies because they can be only segment with positive findings. Likewise in presence of exclusive electromyographic alterations at paraspinal muscles, we should consider GAA enzyme deficiency.
No. 40 Peripheral and Trigeminal Neuropathy in Sickle Cell Anemia: Correlation With Disease Severity and Radiological Findings. Naglaa Ai Gadallah; Hanan El Sebaie El Hefnawy; Sahar Fathi Ahmed; Amel Fahmy Mahdy-Jehan Goda Ali. Disclosure: None. Introduction: Sickle cell anemia (SCA) is the most common genetic disease of the blood. Sickle cell vaso-occlusive crisis (VOC) may cause painful peripheral neuropathy (PN) due to nerve
PRESENTATIONS
ischemia or infarction. The mandible is the most vulnerable area of the face because of relatively low blood flow. Objective: To assess electrophysiologically the possibility of associated subclinical PN or trigeminal neuropathy in SCA patients and correlate results with disease severity and computed tomography (CT) findings. Methods: Fifty patients with SCA were included. Twenty of them had symptoms of PN or trigeminal neuropathy while the others are asymptomatic. 40 matched healthy individuals served as controls. Nerve conduction studies and somatosensory evoked potentials were performed for upper and lower extremities. Trigeminal assessment was performed using trigeminal evoked potential and inferior alveolar nerve (IAN) sensory conduction studies. Findings were correlated with disease severity and CT measured mental foramen and mandibular canal dimensions. Results: Significant decay of the amplitudes of motor and sensory responses of peripheral nerves was detected. There were highly significantly longer trigeminal N13 19 20 latencies and reduced N13-P19 amplitude in patients groups in comparison to controls. There were delayed P19 latency IAN latency reduced IAN amplitude and conduction velocity in SCA patients with clinical trigeminal neuropathy versus asymptomatic ones and also in asymptomatic patients versus controls. These parameters were correlated with frequency of VOC mental foramen and mandibular canal dimensions in SCA patients with trigeminal neuropathy. Conclusions: Subclinical PN and trigeminal neuropathy may be associated with SCA. Trigeminal nerve could be affected along its peripheral or central pathway. Central affection may occur as a result of lesions in its nuclei or at the somatosensory cortex. Electrophysiological assessment is recommended in SCA patients to diagnose and detect the level of trigeminal neuropathy. This will provide new insights into its prevention and treatment.
No. 41 Axonal Sensory-Motor Polyneuropathy on Rheumatoid Arthritis. A Case Report. González Sánchez Mariana; Gómez Toledo Carmina; Aceves Dávalos Ana Eloina Presno Rubín; Diana Helena. Disclosure: None. Setting: Tertiary care general hospital. Patient: A 60year-old female with rheumatoid arthritis (RA). Case Description: The patient was diagnosed with rheumatoid arthritis 20 years ago and has had irregular treatment since then. She started with symmetric progressive general weakness predominantly in proximal muscles. It progressed until it impaired the gait, deteriorating her functional status from class II to IV on the global functional status in RA. At the clinical examination we observed normal muscle tone superficial and deep, sensibility tests were normal, and muscle strength measure by the medical research council scale was 2/5 on proximal muscles and 3/5 on distal muscles on all four limbs. Stretch reflexes were decreased on four extremities. She was diagnosed with mixed cardiomyopathy. Blood tests reported lymphocytopenia, hypocomplementemia, muscle enzymes were normal, acute phase reactants and rheumatoid factor were elevated. A nerve conduction study was performed and we found axonal variety alterations on both motor and sensorial components on four limbs. The monopolar needle electromyography reported increased insertional activity with spontaneous activity at rest (fibrillations, positive sharp waves and complex repetitive discharges), motor unit action potentials were polyphasic and we encounter delayed recruitment pattern. The salivary glands biopsy for Sjögren syndrome was reported negative. Assessments/Results: The patient was diagnosed with axonal sensory-motor polyneuropathy and was treated with corticosteroids and disease-modifying antirheumatic drugs without showing improvement. Discussion: This case confirmed what is mentioned on the reviewed bibliography for this article that peripheral neuropathy is more common in patients with more than ten years since diagnosed with RA and with deteriorating functional status. Conclusions: Axonal polyneuropathies can be a cause of weakness and disability in patients with RA. An early diagnosis and physical and medical therapy can help prevent progression of the disability.
be highly sensitive and specific in the same way should reduce the number of tests that are used to support a diagnosis of CTS. Methods: 89 patients with a diagnosis of CTS were evaluated. Antidromic median palmar sensory latency (APMSL) was recorded in the palm using an electrode over the motor point of the second lumbrical interossei muscle. Median nerve electrical stimulation was performed using a fixed distance of 8 cm proximal to the active recording electrode at the wrist and the peak latency was used. The low filter was 10 Hz, high filter 2 kHz, sweep duration 2 ms, and sensitivity 20 mv. The abnormal cutoff based upon 168 normal controls was: 2.1 ms (range 1.4-2.1 mean 1.8 SD 0.1). Patients with severe conductions delays were not included. Routine motor and sensory distal latency tests of the median and ulnar nerves were also made. Results: Sensitivity and specificity were 81% and 96%; positive and negative predictive values were 91% and 90%. Summary/ conclusion: To our knowledge there is not a previous description of the proposed new technique. The accuracy of this test in confirming cases of CTS was good. It is a fast, easy to perform test in the diagnosis of CTS and consistently found to be highly sensitive and specific.
No. 39 Electromyographic Alterations in Paraspinal Muscles as Unique Finding in a Patient With Deficiency of Alpha Glucosidase. Pedro A. Escobar, MD; Alvaro E. Rodriguez, MD; Edicson Ruiz; Fernando Ortiz. Disclosure: None. Setting: Orthopedic rehabilitation center in Bogota, Colombia. Patient: Male, 57 years old. Case Description: 7 years evolution of gait difficulties principally in sloping ground but also in climbing stairs and to achieve bipedestation from sitting position. He denied respiratory symptoms, dysphagia or sphincter incontinence. Physical examination showed weakness at shoulder and pelvic girdles. Stretch reflexes were normal and symmetrical. Assessment/Results: Electromyography (EMG) of lower thoracic (below T8 level) and lumbar paraspinal muscles showed membrane instability signs, complex repetitive discharges, fibrillation potentials, and positive sharp waves. Quantitative EMG by interference pattern analysis and motor unit action potentials (MUAP) were normal in quadriceps, gluteus medius and gluteus maximus muscles. Creatine phosphokinase (CPK) 427. Magnetic resonance images (MRI) of brain showed white matter microangiopathy. Lumbar MRI showed atrophy of paraspinal muscles. Acid alpha-glucosidase (GAA) levels: 0.3 mmol/l/h. (normal values > 39 umol/l/h) and double mutation in GAA gene (c.-32-13t>g y c.887del cp.p296lfs*18). Discussion: Disease by deficiency of GAA enzyme produces progressive muscle disorders which leads to abnormal buildup of intracellular glycogen principally in skeletal cardiac and smooth muscles. It leads to weakness, organic failure, and consequently death. Weakness severity is variable because of degree of enzymatic deficiency displaying a wide spectrum of clinical presentation. EMG occasionally shows alterations in paraspinal muscles only. Conclusion: Electrophysiological laboratories in case of suspicion of GAA enzyme deficiency should do paraspinal muscles EMG studies because they can be only segment with positive findings. Likewise in presence of exclusive electromyographic alterations at paraspinal muscles, we should consider GAA enzyme deficiency.
No. 40 Peripheral and Trigeminal Neuropathy in Sickle Cell Anemia: Correlation With Disease Severity and Radiological Findings. Naglaa Ai Gadallah; Hanan El Sebaie El Hefnawy; Sahar Fathi Ahmed; Amel Fahmy Mahdy-Jehan Goda Ali. Disclosure: None. Introduction: Sickle cell anemia (SCA) is the most common genetic disease of the blood. Sickle cell vaso-occlusive crisis (VOC) may cause painful peripheral neuropathy (PN) due to nerve
PRESENTATIONS
ischemia or infarction. The mandible is the most vulnerable area of the face because of relatively low blood flow. Objective: To assess electrophysiologically the possibility of associated subclinical PN or trigeminal neuropathy in SCA patients and correlate results with disease severity and computed tomography (CT) findings. Methods: Fifty patients with SCA were included. Twenty of them had symptoms of PN or trigeminal neuropathy while the others are asymptomatic. 40 matched healthy individuals served as controls. Nerve conduction studies and somatosensory evoked potentials were performed for upper and lower extremities. Trigeminal assessment was performed using trigeminal evoked potential and inferior alveolar nerve (IAN) sensory conduction studies. Findings were correlated with disease severity and CT measured mental foramen and mandibular canal dimensions. Results: Significant decay of the amplitudes of motor and sensory responses of peripheral nerves was detected. There were highly significantly longer trigeminal N13 19 20 latencies and reduced N13-P19 amplitude in patients groups in comparison to controls. There were delayed P19 latency IAN latency reduced IAN amplitude and conduction velocity in SCA patients with clinical trigeminal neuropathy versus asymptomatic ones and also in asymptomatic patients versus controls. These parameters were correlated with frequency of VOC mental foramen and mandibular canal dimensions in SCA patients with trigeminal neuropathy. Conclusions: Subclinical PN and trigeminal neuropathy may be associated with SCA. Trigeminal nerve could be affected along its peripheral or central pathway. Central affection may occur as a result of lesions in its nuclei or at the somatosensory cortex. Electrophysiological assessment is recommended in SCA patients to diagnose and detect the level of trigeminal neuropathy. This will provide new insights into its prevention and treatment.
No. 41 Axonal Sensory-Motor Polyneuropathy on Rheumatoid Arthritis. A Case Report. González Sánchez Mariana; Gómez Toledo Carmina; Aceves Dávalos Ana Eloina Presno Rubín; Diana Helena. Disclosure: None. Setting: Tertiary care general hospital. Patient: A 60year-old female with rheumatoid arthritis (RA). Case Description: The patient was diagnosed with rheumatoid arthritis 20 years ago and has had irregular treatment since then. She started with symmetric progressive general weakness predominantly in proximal muscles. It progressed until it impaired the gait, deteriorating her functional status from class II to IV on the global functional status in RA. At the clinical examination we observed normal muscle tone superficial and deep, sensibility tests were normal, and muscle strength measure by the medical research council scale was 2/5 on proximal muscles and 3/5 on distal muscles on all four limbs. Stretch reflexes were decreased on four extremities. She was diagnosed with mixed cardiomyopathy. Blood tests reported lymphocytopenia, hypocomplementemia, muscle enzymes were normal, acute phase reactants and rheumatoid factor were elevated. A nerve conduction study was performed and we found axonal variety alterations on both motor and sensorial components on four limbs. The monopolar needle electromyography reported increased insertional activity with spontaneous activity at rest (fibrillations, positive sharp waves and complex repetitive discharges), motor unit action potentials were polyphasic and we encounter delayed recruitment pattern. The salivary glands biopsy for Sjögren syndrome was reported negative. Assessments/Results: The patient was diagnosed with axonal sensory-motor polyneuropathy and was treated with corticosteroids and disease-modifying antirheumatic drugs without showing improvement. Discussion: This case confirmed what is mentioned on the reviewed bibliography for this article that peripheral neuropathy is more common in patients with more than ten years since diagnosed with RA and with deteriorating functional status. Conclusions: Axonal polyneuropathies can be a cause of weakness and disability in patients with RA. An early diagnosis and physical and medical therapy can help prevent progression of the disability.