How Long does Denervation Take in Poliomyelitis? Or is it a Lifetime?”

ABSTRACT Background and Objective: This study aims to determine the period of reinnervation in patients with poliomyelitis. This research was conducted to identify the appearance of denervation potentials in patients with poliomyelitis as indicators for reinnervation. Materials and Methods: A total of 246 male patients with poliomyelitis were assessed electrophysiologically between 1988 and 2007. The mean age was 22.8 (18±42). It has been an average of 19.9 ± 4.9 years since the beginning of complaints from the patients. Results: The patients had no complaints of newly developing muscle weakness, fatigue, muscle and joint pain, and difficulties in breathing and swallowing. Neurological examinations revealed the absence of myotomal pain and sensory loss. Upon assessment of the patients' limbs, the following findings were revealed: two patients had left upper and lower limb involvement, two patients had left upper and right lower limb involvement, 6 patients had left upper limb involvement, 12 patients had both lower limb involvement, 105 patients had left lower limb involvement, 1 patient had both upper limb involvement, 2 patients had right lower and upper limb involvement, 12 patients had right upper limb involvement, 6 patients had both lower limb involvement, 95 patients had right lower limb involvement, and 3 had all the three extremities affected. The needle electromyography revealed the presence of denervation potentials in 25.2% (62) of the patients. Conclusion: When poliovirus attacks the motor neuron, this neuron may be completely destroyed, damaged, or unaffected. Reinnervation occurs when nearby functioning motor units send out terminal axon sprouts to reinnervate the damaged muscle fibers. As a consequence of poliomyelitis, several muscle fibers become atrophic and fibrotic, but others continue to survive. This study showed that patients with a history of poliomyelitis experienced denervation with subsequent reinnervation for many years.

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Relationships between Upper Limb, Lower Limb and Trunk Muscle Mass and Insulin Resistance in Male Patients with Type 2 Diabetes

Rigakuryoho Kagaku ◽

10.1589/rika.35.735 ◽

2020 ◽

Vol 35 (5) ◽

pp. 735-739

Author(s):

Yoshikazu HIRASAWA ◽

Ryosuke MATSUKI ◽

Hideaki TANINA ◽

Toshihiko EBISU ◽

Yoshiyuki HAMAMOTO

Keyword(s):

Insulin Resistance ◽

Type 2 Diabetes ◽

Muscle Mass ◽

Upper Limb ◽

Lower Limb ◽

Trunk Muscle ◽

Male Patients

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SOD1 Mutation Spectrum and Natural History of ALS Patients in a 15-Year Cohort in Southeastern China

Frontiers in Genetics ◽

10.3389/fgene.2021.746060 ◽

2021 ◽

Vol 12 ◽

Author(s):

Lu-Xi Chen ◽

Hai-Feng Xu ◽

Pei-Shan Wang ◽

Xin-Xia Yang ◽

Zhi-Ying Wu ◽

...

Keyword(s):

Clinical Trials ◽

Natural History ◽

Diagnostic Delay ◽

Mutation Spectrum ◽

Survival Duration ◽

Southeastern China ◽

History Of ◽

Male Patients ◽

The Mean ◽

Als Patients

Background: Mutations in superoxide dismutase 1 gene (SOD1) are the most frequent high penetrant genetic cause for amyotrophic lateral sclerosis (ALS) in the Chinese population. A detailed natural history of SOD1-mutated ALS patients will provide key information for ongoing genetic clinical trials.Methods: We screened for SOD1 mutations using whole exome sequencing (WES) in Chinese ALS cases from 2017 to 2021. Functional studies were then performed to confirm the pathogenicity of novel variants. In addition, we enrolled previously reported SOD1 mutations in our centers from 2007 to 2017. The SOD1 mutation spectrum, age at onset (AAO), diagnostic delay, and survival duration were analyzed.Results: We found two novel SOD1 variants (p.G17H and p.E134*) that exerted both gain-of-function and loss-of-function effects in vitro. Combined with our previous SOD1-mutated patients, 32 probands with 21 SOD1 mutations were included with the four most frequently occurring mutations of p.V48A, p.H47R, p.C112Y, and p.G148D. SOD1 mutations account for 58.9% of familial ALS (FALS) cases. The mean (SD) AAO was 46 ± 11.4 years with a significant difference between patients carrying mutations in exon 1 [n = 5, 34.6 (12.4) years] and exon 2 [n = 8, 51.4 (8.2) years] (p = 0.038). The mean of the diagnostic delay of FALS patients is significantly earlier than the sporadic ALS (SALS) patients [9.5 (4.8) vs. 20.3 (9.3) years, p = 0.0026]. In addition, male patients survived longer than female patients (40 vs. 16 months, p = 0.05).Conclusion: Our results expanded the spectrum of SOD1 mutations, highlighted the mutation distribution, and summarized the natural history of SOD1-mutated patients in southeastern China. Male patients were found to have better survival, and FALS patients received an earlier diagnosis. Our findings assist in providing a detailed clinical picture, which is important for ongoing genetic clinical trials.

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Further evidence of incomplete neural control of muscle properties in cat tibialis anterior motor units

AJP Cell Physiology ◽

10.1152/ajpcell.1995.268.2.c527 ◽

1995 ◽

Vol 268 (2) ◽

pp. C527-C534 ◽

Cited By ~ 13

Author(s):

G. A. Unguez ◽

R. R. Roy ◽

D. J. Pierotti ◽

S. Bodine-Fowler ◽

V. R. Edgerton

Keyword(s):

Motor Unit ◽

Tibialis Anterior ◽

Neural Control ◽

Periodic Acid ◽

Muscle Fibers ◽

Motor Units ◽

Fiber Types ◽

Muscle Properties ◽

Periodic Acid Schiff ◽

The Mean

To examine the influence of a motoneuron in maintaining the phenotype of the muscle fibers it innervates, myosin heavy chain (MHC) expression, succinate dehydrogenase (SDH) activity, and cross-sectional area (CSA) of a sample of fibers belonging to a motor unit were studied in the cat tibialis anterior 6 mo after the nerve branches innervating the anterior compartment were cut and sutured near the point of entry into the muscle. The mean, range, and coefficient of variation for the SDH activity and the CSA for both motor unit and non-motor unit fibers for each MHC profile and from each control and each self-reinnervated muscle studied was obtained. Eight motor units were isolated from self-reinnervated muscles using standard ventral root filament testing techniques, tested physiologically, and compared with four motor units from control muscles. Motor units from self-reinnervated muscles could be classified into the same physiological types as those found in control tibialis anterior muscles. The muscle fibers belonging to a unit were depleted of glycogen via repetitive stimulation and identified in periodic acid-Schiff-stained frozen sections. Whereas muscle fibers in control units expressed similar MHCs, each motor unit from self-reinnervated muscles contained a mixture of fiber types. In each motor unit, however, there was a predominance of fibers with the same MHC profile. The relative differences in the mean SDH activities found among fibers of different MHC profiles within a unit after self-reinnervation and those found among fibers in control muscles were similar, i.e., fast-2 < fast-1 < or = slow MHC fibers.(ABSTRACT TRUNCATED AT 250 WORDS)

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Water Reductive Effect of Lymphaticovenular Anastomosis on Upper-Limb Lymphedema: Bioelectrical Impedance Analysis and Comparison with Lower-Limb Lymphedema

Journal of Reconstructive Microsurgery ◽

10.1055/s-0040-1713670 ◽

2020 ◽

Vol 36 (09) ◽

pp. 660-666 ◽

Cited By ~ 1

Author(s):

Yoshichika Yasunaga ◽

Daisuke Yanagisawa ◽

Yuta Nakajima ◽

Shinei Mimura ◽

Miharu Kobayashi ◽

...

Keyword(s):

Upper Limb ◽

Lower Limb ◽

Bioelectrical Impedance Analysis ◽

Reduction Rate ◽

Bioelectrical Impedance ◽

Impedance Analysis ◽

Volume Reduction ◽

Water Volume ◽

Arm Lymphedema ◽

The Mean

Abstract Background We previously examined the water reductive effect of lymphaticovenular anastomosis (LVA) using bioelectrical impedance analysis (BIA) measurement on lower-limb lymphedema and revealed mean water volume reduction and edema reduction rate by leg LVA to be 0.86 L and 45.1%, respectively. This study aimed to clarify the water reductive effect of LVA on arm lymphedema and compare its results with those for leg lymphedema. Patients and Methods The efficacy of LVA for unilateral arm lymphedema was evaluated using BIA in a retrospective cohort. Limb circumference and arm body water volume (ABW) of the affected and unaffected arms were measured before and after LVA. Mean water volume reduction (ΔABW) and edema reduction rate by arm LVA were compared with values for leg LVA cited from our previous report as a historical control. Results Nineteen consecutive patients were enrolled. The mean ΔABW and edema reduction rate by BIA were 0.267 L and 46.0%, respectively. The decreasing rate of ABW by BIA was significantly larger than those of the upper extremity lymphedema index and sum of 5 circumferences measurement methods. ΔABW could be predicted by a regression line based on the preoperative water volume difference between affected and unaffected limbs. The mean edema reduction rates for arm and leg LVA were comparable. Conclusion The water reductive effect of LVA on upper-limb lymphedema was demonstrated by BIA assessment. BIA can reflect the effect of LVA more sensitively than conventional objective measurements and may facilitate the interpretation of LVA results. Although water volume reduction by arm LVA was less than that by leg LVA, the edema reduction rates were comparable.

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Selected Profile of Psychiatric Patients Admitted in a Specialized Hospital

KYAMC Journal ◽

10.3329/kyamcj.v7i2.33836 ◽

2017 ◽

Vol 7 (2) ◽

pp. 770-774

Author(s):

Tanmay Prakash Biswas ◽

Buran Uddin Haider ◽

Saroj Kumar Dass

Keyword(s):

Mental Hospital ◽

Psychiatric Patients ◽

Positive Family History ◽

Age Group ◽

Specialized Hospital ◽

History Of ◽

Group Population ◽

Male Patients ◽

The Mean ◽

Time Of Admission

The current study had been carried out on the admitted patients in Mental Hospital, Pabna who was admitted during the calendar year 2008. Mental patients having only psychotic type (major) of illness are admitted in this hospital. A total of 101 (one hundred and one) admitted patients were included in the study. Among them, male patients were 79 (78.22%) and females were 22 (21.78%). Their age ranges from 19 (nineteen) to 48 (forty eight). The mean age of the patients was 27.35. Among the respondents, 21 to 30 year age group population found suffering more (46.54%) in this study than other age group. The number of the first group people is a few which may be due to the small age span. More than half (55.45%) of the cases were unmarried. Secondary and Higher secondary educated people occupies more than half (57.43%) of the respondents. Illiterate group population occupies the third (12.87%) position. More than half ( 55.45%) of the cases do not have any job during the time of admission. About two-thirds (63.37%) of the cases were from rural background and more than half (58.42%) were diagnosed as Schizophrenics. Schizo- affective disorder (25.74%) occupies the second position so far the diagnosis of the respondents were concerned. Most of them (81.19%) did not give any positive family history of mental illness. More than two-thirds (66.34%) of the cases have given the history of taking mixed type of treatment before attending the OPD of Mental hospital, Pabna.KYAMC Journal Vol. 7, No.-2, Jan 2017, Page 770-774

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Epidemiology of human brucellosis in Nahavand county, Hamadan Province, western Iran: an 8-year (2010–2017) registry-based analysis

Asian Biomedicine ◽

10.1515/abm-2020-0022 ◽

2020 ◽

Vol 14 (4) ◽

pp. 151-158

Author(s):

Salman Khazaei ◽

Manoochehr Solgi ◽

Shahram Goodarzi ◽

Leila Khazaei ◽

Iraj Salehi ◽

...

Keyword(s):

Rural Areas ◽

Human Brucellosis ◽

Male Population ◽

Female Population ◽

Western Iran ◽

History Of ◽

Male Patients ◽

The Mean ◽

Rural Dwellers ◽

Epidemiological Characteristics

AbstractBackgroundBrucellosis is an infectious zoonosis, which greatly afflicts human health and animal productivity.ObjectivesTo describe the trends and epidemiological characteristics of human brucellosis in Nahavand county, Hamadan Province, western Iran over 8 years (2010–2017).MethodsIn this registry-based longitudinal study, we analyzed all reported cases of human brucellosis, including 1,130 patients from 2010 to 2017. A checklist including demographic and clinical characteristics of patients with reported cases of brucellosis had been used to gather data. We calculated annual percent changes (APCs) and average annual percent changes (AAPCs) using Joinpoint software to determine the trend of brucellosis using a segmented regression model.ResultsAmong the patients, 60.9% were male and 87.3% lived in rural areas. The mean age of the patients was 35.9 ± 18.34 years. Compared with male patients, female patients were more affected when they were elderly (15.6% men vs. 24.2% women for those aged ≥55 years) (P = 0.001). Of patients with brucellosis, 65.2% had consumed unpasteurized dairy products and 82.3% had a history of contact with animals. The incidence of human brucellosis in Nahavand county increased between 2010 and 2014, then decreased in 2015, and thereafter remained steady. Per 100,000 population, the AAPC pertaining to the incidence was 17.4 in the male population (95% CI: 4.4, 31.9), 13.8 in the female population (95% CI: 0.2, 29.3), and 16.1 in rural dwellers (95% CI: 2.2, 31.8) indicating an increasing trend from 2010 to 2017 (P < 0.05).ConclusionThe incidence of brucellosis in the western part of Iran is high and remains a challenging health problem. In the present study, age, job, sex, and seasonal changes are important risk factors for human brucellosis.

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Effect of compensatory hypertrophy studied in individual motor units in medial gastrocnemius muscle of the cat

Journal of Neurophysiology ◽

10.1152/jn.1978.41.2.496 ◽

1978 ◽

Vol 41 (2) ◽

pp. 496-508 ◽

Cited By ~ 30

Author(s):

J. V. Walsh ◽

R. E. Burke ◽

W. Z. Rymer ◽

P. Tsairis

Keyword(s):

Motor Unit ◽

Muscle Fibers ◽

Motor Units ◽

Compensatory Hypertrophy ◽

Medial Gastrocnemius ◽

Cross Sectional ◽

Unit Type ◽

The Mean ◽

Tetanic Tension ◽

Individual Motor

1. Compensatory hypertrophy of the medial gastrocnemius (MG) muscle was produced by denervating or removing its synergists (i.e., the lateral gastrocnemius, soleus, and plantaris muscles) in adult cats. Following survival times of 14-32 wk, intracellular recording and stimulation techniques were used to study the motor-unit population in MG. The data obtained were compared with results from MG motor units in normal unoperated cats of the same body size and weight. 2. Using criteria employed for normal motor units, the units in hypertrophic MG muscles were readily classified into the same groups (types FF, F(int), FR, and S) as in normal MG. There was no detectable difference in the distribution of motor-unit types after hypertrophy. 3. When compared with a normal motor-unit sample, there was a large increase in mean tetanic tension, but no significant change in twitch tension, for each motor-unit type in the hypertrophied muscles. The most marked increase was found among the fatigue-resistant type S and type FR motor units. There was no alteration of twitch contraction times or fatigue resistance in any unit type after hypertrophy. 4. For each motor-unit type, the mean homonymous (MG) group Ia EPSP amplitude was the same in normal and hypertrophic MG populations. There was, however, a significant increase in the average conduction velocity of MG motor axons in the animals with uncomplicated MG synergist removal and maximal MG hypertrophy. 5. On the basis of histochemical staining, muscle fibers from comparable sections of hypertrophic and contralateral (unoperated) MG muscles were presumptively identified as belonging to FF, FR, or S units. There was no significant difference between hypertrophic and contralateral MG muscles in the percentage of each fiber type, although there was some variability in muscle composition from one cat to another. One muscle pair was studied in detail for fiber cross-sectional area. In this cat, with marked hypertrophy by muscle weight, there was a modest increase in the mean fiber areas of histochemical S and FR muscle fibers, but no evident change in FF fibers, on the hypertrophic side. 6. MG motor units were examined in several cats in which synergist removal resulted in scarring and marked limitation of passive ankle mobility, and no evident weight gain in MG. Motor units of all types in these animals showed a decrease in twitch tension and in mean twitch/tetanus ratios, with little alteration in mean tetanic tensions. 7. The main effect of compensatory hypertrophy under the present conditions was a large increase in tetanic tension output from individual motor units due, at least in part, to an increase in fiber cross-sectional area. There was no evidence indicating any "conversion" of motor units or of their muscle fibers from one type to another.

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Cervical extradural metastasis from follicular carcinoma thyroid after 14 years post-thyroidectomy with Elsberg phenomenon

Romanian Neurosurgery ◽

10.33962/roneuro-2020-095 ◽

2020 ◽

pp. 557-560

Author(s):

Vijayan Pettakandy ◽

Shanavas Cholakkal ◽

Subrat Kumar Soren ◽

Harikrishnan S.

Keyword(s):

Upper Limb ◽

Lower Limb ◽

Thyroid Tissue ◽

Follicular Carcinoma ◽

Lower Limbs ◽

Sensory Loss ◽

Cervical Region ◽

Left Lower Limb ◽

Grade 3 ◽

Follicular Carcinoma Thyroid

Background. Follicular carcinoma thyroid usually metastasises to bone. Common sites of bone metastasis include skull and spine. Spinal metastasis are more common in the cervical region followed by dorsolumbar spine. Cervical extradural lesions present with progressive quadriparesis, sensory loss, dysautonomia, and respiratory distress. Typical Elsberg phenomenon in a cervical extradural lesion is rare. Elsberg phenomenon involves the involvement of ipsilateral upper limb, ipsilateral lower limb followed by contralateral lower limb and contralateral upper limb. Case presentation. We are reporting a case of 47-year-old lady presented with progressive quadriparesis of 1-month duration. Her weakness started in left upper limb followed by left lower limb, right lower limb and right upper limb weakness. She also had sensory loss below the level of C7. She had undergone near-total thyroidectomy for solitary thyroid nodule 14 years back and was on thyroid supplementation since then. Histopathology at that time was reported as follicular adenoma with Hashimoto thyroiditis. Her right upper limb power was grade 4- Left upper limb grade 1 right lower limb Grade 3, left lower limb grade 2 with hypertonia of both upper and lower limbs. She was evaluated with MRI Spine which showed a dumb bell-shaped extramedullary lesion involving the C5-C6 vertebra with significant cord compression and encasement of the left vertebral artery. USG neck showed left supraclavicular lymph node enlargement and small residual thyroid tissue in the left side of the thyroid. USG guided FNAC from the thyroid tissue and neck nodes were inconclusive. The patient underwent C4 and C5 laminectomy and subtotal excision from the cervical lesion. Histopathology was reported as metastasis from follicular carcinoma thyroid. Postoperatively patient limb power improved to grade 3 left upper and lower limbs and was discharged and later referred for radioiodine ablation Conclusion. Cervical extradural metastasis from follicular carcinoma thyroid can present with Elsberg syndrome even without any neck swelling even after decades of post thyroidectomy status for a benign aetiology. Laminectomy and decompression may lead to clinical improvement.

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105 Snake eyes in the thoracic spine

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-anzan.104 ◽

2018 ◽

Vol 89 (6) ◽

pp. A41.3-A42

Author(s):

Joel Corbett ◽

Stephen Walsh ◽

Sandeep Bhuta ◽

Arman Sabet

Keyword(s):

Motor Neuron ◽

Motor Neuron Disease ◽

Upper Limb ◽

Thoracic Spine ◽

Lower Limb ◽

Anterior Horn ◽

Lower Motor Neuron ◽

Anterior Horn Cell ◽

Motor Neuron Diseases ◽

History Of

IntroductionBilateral anterior horn cell hyperintensity on spinal imaging (‘snake eyes’ sign) is seen in pathologies including cervical spondylosis, spinal cord infarction and Hirayama’s disease. Below is the first report of lower limb monomelic amyotrophy (MMA) with thoracic spine snake eyes sign. We present a case report of lower limb MMA with bilateral anterior horn hyperintensity, and literature review of cases with this clinico-radiologic presentation.CaseA 47 year old man presented with an 11 year history of asymmetric, progressive, proximal right lower limb weakness and wasting following traumatic back injury. Eight years into the disease course left leg changes developed. There was no upper limb, bulbar nor respiratory involvement. Examination revealed widespread lower limb wasting, normal tone, marked proximal weakness, brisk reflexes and non-sustained clonus bilaterally. Upper limb and cranial nerve examinations were normal. MRI demonstrated T11–12 bilateral anterior horn cell hyper-intensity. Electromyography demonstrated denervation/re-innervation changes in the right vastus lateralis and to a lesser extent tibialis anterior. Muscle biopsy showed chronic denervation atrophy. Anti-ganglioside GM1 IgM was elevated. Further autoimmune testing, infectious screen, cerebrospinal fluid and neuromuscular disease gene analysis were negative. Steroid and intravenous immunoglobulin therapy were ineffective.Case series describing lower motor neuron diseases (LMND) including MMA have not previously reported snake eyes sign in association with lower limb disease.1 Two recent publications describing thirty-two cases of LMND with cervical spine snake eyes sign report that all cases were associated with a relatively benign course and many were misdiagnosed as motor neuron disease (MND).2 3 The authors propose this as a previously unidentified mimic of motor neuron disease.ConclusionThis is the first reported case of thoracic snake eyes sign with corresponding lower limb MMA. Lower motor neuron diseases with bilateral anterior horn cell hyper-intensity may represent a unique clinical form of MND with relatively slower progression.References. Nalini A, Gourie-Devi, Thennarasu K, et al. Monomelic Amyotrophy: Clinical profile and natural history of 279 cases seen over 35 years (1976–2010). Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration2014;15:457–465.. Saski S. Sporadic lower motor neuron disease with a snake eyes appearance on the cervical anterior horns by MRI. Clinical Neurology and Neurosurgery2015;136:122–131.. Lebouteux M, Franques J, Guillevin R, et al. Revisiting the Spectrum of Lower Motor Neuron Diseases with Snake Eyes Appearance on Magnetic Resonance Imaging. European Journal of Neurology2014;21:1233–1241.

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Differences in estimated persistent inward currents between ankle flexors and extensors in humans

Journal of Neurophysiology ◽

10.1152/jn.00746.2019 ◽

2020 ◽

Vol 124 (2) ◽

pp. 525-535

Author(s):

Edward H. Kim ◽

Jessica M. Wilson ◽

Christopher K. Thompson ◽

Charles J. Heckman

Keyword(s):

Upper Limb ◽

Lower Limb ◽

Tibialis Anterior ◽

Motor Units ◽

Separation Algorithm ◽

Persistent Inward Currents ◽

Future Studies ◽

Inward Currents ◽

Density Surface ◽

Convolutive Blind Source Separation

With the use of high-density surface array electrodes and convolutive blind source separation algorithm, thousands of motor units were decomposed from the soleus and tibialis anterior muscles. Persistent inward currents were estimated under seated and standing conditions via delta-F (∆F) calculation, and the results showed that unlike the upper limb, the flexor has higher ∆F than the extensor in the lower limb. Future studies will focus on functional significance of the findings.

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