Fiber type and fiber size of cat ankle, knee, and hip extensors and flexors following low thoracic spinal cord transection at an early age

Effects of low thoracic spinal transection on muscle weights, fiber type distributions, and fiber cross-sectional areas (CSAs) of selected cat hind limb mixed-fast flexors and extensors and slow extensors were studied. Cats were spinalized at T12 at 2 weeks of age and maintained for 6 to 12 months. In general spinalization resulted in a decrease in muscle weights of extensors, while the weights of those muscles that function as either flexors or as both flexors and extensors were maintained. The percentages of fast-twitch [fast glycolytic (FG) and fast oxidative-glycolytic (FOG)] fibers increased and slow oxidative (SO) fibers decreased as a result of spinalization. However, FG fibers had a smaller CSA after spinal transection in both extensors and flexors. The total relative CSA of FG fibers per whole muscle was similar in spinalized and control cats. The relative muscle CSA occupied by SO fibers decreased in extensors and flexors as a result of a lower proportion of SO fibers and/or smaller SO fibers in spinalized cats. These findings suggest that muscles become more "fast-like" histochemically while little change occurs in the oxidative staining properties in either extensors or flexors in 6- to 12-month-old cats transected at 2 weeks of age. Further, these data suggest that the amount of muscle atrophy that occurs as a result of spinal transection is not closely related to the percentage reduction in SO fibers.     

A manifesting carrier of Duchenne muscular dystrophy presenting mosaic distribution of dystrophin negative and positive muscle fibers

A 25-year-old female patient with an approximate 10-year-history of slowly progressive muscle weakness was diagnosed as a manifesting carrier of Duchenne muscular dystrophy (DMD) because her muscle biopsy showed scattered fibers with no dystrophin on immunohistochemical staining. She had no family history of neuromuscular disorders. She was in good health until about 14 years of age, when she developed muscle weakness and atrophy of the extremities with slow aggravation. On admission at the age of 25 years, she had asymmetrical muscle atrophy in the lower extremities; the left femur, right femur, left crus, and right crus measured 36.0, 40.5, 31.5, and 35.5 cm in circumference, respectively. However, the muscle weakness of the extremities was symmetrical with no laterality, and the proximal muscles in the lower extremities were predominantly affected to 3+/5 MMT test. She walked with a mild wadding manner and stood up with Gower' maneuver. Deep tendon reflexes of the extremities were almost normoactive with no pathologic reflexes. As to laboratory findings, serum enzymes of muscular origin were elevated; GOT was 44 IU/l, GPT 60 IU/l, LDH 829 IU/l, CK 4238 IU/l, and aldolase 31 SL units. The electromyogram showed myopathic changes mixed with some neurogenic components. Peripheral nerve conduction velocity was normal. A computed tomography of the skeletal muscles showed more marked atrophy and lower density in the left lower extremity than in the right. The biopsied left gastrocnemius muscle demonstrated a marked variation in fiber size with some necrotic and regenerating fibers. On immunohistochemical stain with anti-dystrophin antibody, the dystrophin negative fibers were scattered among positive fibers in a mosaic distribution.     

HTLV-I associated encephalo-myelopathy resembling ALS with concurrence of acute promyelocytic leukemia in a member of the relatives

A 36 year-old woman beginning with spastic paraparesis at her age of 11 visited us for evaluation of progressive muscular weakness of the distal portions of the upper extremities and difficulty in speaking at her age of 33. The neurological features at the present are as follows; fine horizontal gaze-nystagmus, impaired smooth pursuit ocular movement, highly spastic paraplegia with pes equino-varus necessitating canes and the wheel-chair, highly accentuated PTRs and ATRs associated with positive Babinski's sign, diminished or absent deep reflexes in the upper extremities, moderate muscular wasting with fasciculation on the tongue and distal portions of the upper extremities (rt less than lt). Sensory or cerebellar functions remain normal. No autonomic finding has been manifested. The HTLV-I antibody titers of serum (eg. PA method: x8192 ) and cerebrospinal fluid are highly positive in various methods. That of her mother (no blood-transfusion history) is positive. The provirus genome analysis on peripheral lymphocytes using the Southern blotting method by the cleaving enzyme Psi I was unable to discriminate that of an ATL patient. MRI of the central nervous system revealed higher signal area (short SE) at the C5/6 region and atrophy of C7/8 region. Neither a definite lesion in the lower brain stem, cerebellum nor cerebral hemispheres was identified. The skeletal muscle X-ray CT on the lower extremities revealed the atrophic flexor thighs and the anterior tibial and peroneal muscles. Needle EMG showed the prominent neurogenic changes in the atrophic muscles. Oral prednisolone therapy for four months relieved nystagmus and difficulty in walking, slightly. She, however, discontinued because of its side effect.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of severe infantile form of congenital nemaline myopathy with extensive fatty replacement of the skeletal muscles

A case of severe infantile form of congenital nemaline myopathy who developed extensive fatty replacement of the skeletal muscles was described. A girl was born with severe hypotonia and flaccidity of the extremities. She was put on a ventilator because of the severe respiratory insufficiency. Muscle biopsy performed at 3 months of age revealed numerous nemaline rods in myofibers. She had an anoxic episode at 2 years of age and fell into a vegetative state after that. Serum creatine kinase and aldolase levels were normal. At 8 years of age, X-ray CT scan of the skeletal muscles revealed diffuse and severe fatty replacement of the skeletal muscles of the trunk and extremities; this was far more extensive than in the case of Duchenne muscular dystrophy of similar age. Second muscle biopsy performed in the anterior tibialis muscle at the age of 8 years revealed atrophic muscle fibers and extensive proliferation of connective and fatty tissues. Electron microscopy revealed, numerous rod-containing muscles fibers with severe disorganization and loss of myofilaments. Sural nerve biopsy performed at the same time showed decreased number of large myelinated fibers. Although a possibility could not be excluded completely that the episode of anoxia and chronic debilitation may have contributed to these pathological neuromuscular findings, it was presumed that severe degeneration and fatty replacement of the skeletal muscles progress rapidly after birth in some cases of severe infantile form of congenital nemaline myopathy.     

A case of adult-onset nemaline myopathy (adult-onset rod disease) with distal muscular hypertrophy]

We reported a 40-year-old male with adult-onset nemaline myopathy (adult-onset rod disease) showing muscular hypertrophy of distal limbs. At the age of 25, he noticed thinness of his thighs. Difficulty in climbing stairs slowly progressed from the age of 35. On admission neurological examination revealed muscular weakness and atrophy of proximal limbs and hypertrophy of distal flexors. Normal laboratory tests included serum creatine kinase, myoglobin, aldolase and pyruvate. Electromyography revealed severe neurogenic changes in the right biceps brachial muscle and the right quadriceps muscle, and moderate changes in the right gastrocnemius. Biopsy specimen of the deltoid muscle demonstrated type 1 fiber predominance and type 1 fiber atrophy, and there was small group atrophy and type grouping. Abundant nemaline rods were found mainly in type 1 fibers (81.5%). In order to evaluate hypertrophy of calf muscles, T1-weighted MRI of lower extremity was performed. While transaxial images through mid thigh showed moderate fatty replacement, increased volume and little fatty replacement were found in the mid calf. Therefore, hypertrophy of the calf muscle seemed to be compensative hypertrophy. But in this case neurogenic factors were indicated electromyographically and histologically. These findings may advocate the notion that neurogenic factors involved not only congenital but adult-onset rod disease.     

Spastic wrist flexors are more severely affected than wrist extensors in children with cerebral palsy

Morphological properties of skeletal muscle were compared between wrist flexors and extensors within the same children (n = 8, six females, two males; age range 4 to 9y, median age 7 y) with wrist muscle imbalance secondary to spastic cerebral palsy (CP). Five patients had hemiplegic CP, two diplegic CP, and one patient had tetraplegic CP. Muscle biopsies were taken during either tendon transfer or tendon lengthening procedures. Analyses included distribution of muscle fibre types, fibre sizes, and expression of developmental myosins. Extensor fibre area was significantly greater than flexor fibre area for type 2A fibres and type 2B fibres but not for type 1 fibres. Coefficient of variation (CV) of fibre size for all three fibre types was greater for flexors compared with extensors. The greatest CV was observed for the type 2A fibres in flexors (39.5 [3.6%]). A wide variation was observed for expression of developmental myosin with the magnitude of the expression being greater, but not statistically significant, in flexors compared with extensors (5.4/mm2 vs 0.53/mm2). These data demonstrate that significant secondary myopathy of wrist flexor muscles results from CP.     

X-ray computed tomographic scans of lower limb and trunk muscles in facioscapulohumeral muscular dystrophy]

X-rays computed tomographic (CT) scans of muscles of the lower limbs and the trunk in 14 patients with facioscapulohumeral muscular dystrophy (FSH) were studied. The CT scans showed that the affected muscles were decreased in density and size. The laterality of muscular involvement was sometimes observed. The muscular lesions in the lower limbs showed proximal distribution. In the thigh, the hamstrings were affected first, the adductor muscles second, and then the muscular involvement progressed to the quadriceps femoris muscle. In the lower leg, the gastrocnemius and soleus muscles were relatively spared as compared with the tibialis anterior muscle. In the lumbar girdle, the abdominal muscles were involved first, the gluteal muscles second, the back muscles third, and the psoas major muscle were relatively spared. The muscular weakness of this distribution exacerbated lumbar lordosis. The neck muscles were less affected than those of the lumbar girdle. The CT scans in FSH demonstrated the characteristic pattern of muscular involvement, which differed from the inherited muscular diseases such as Duchenne muscular dystrophy, myotonic dystrophy, and others.     

Quantitative motor assessment in myotonic dystrophy

OBJECTIVE: To establish baseline data, using a quantitative motor evaluation protocol, prior to a prospective longitudinal study of the natural history of muscular involvement in myotonic dystrophy (DM). DESIGN/METHODS: We conducted a cross-sectional study using a protocol consisting of manual muscle testing (MMT), quantitative muscle testing (QMT), and timed functional testing (TFT) on 50 definite DM patients (27 men, 23 women), aged 16 to 67 years. The relationships between MMT, QMT and TFT scores and disease duration were examined using linear regression analysis. RESULTS: The muscle weakness was symmetric and the neck flexors and the distal muscles of upper and lower extremities were weaker than proximal muscles. Using MMT scores, the average strength decline was 0.95% per year and was similar for men and women. The strength decline was significantly more rapid for distal muscles than for proximal muscles. Quantitative muscle testing scores documented a strength decline per year of disease duration of 1.2-1.6% for the hip flexors and of 2.0-3.0% for the hand grip flexors. CONCLUSIONS: We observed significant linear relationships between the scores generated by this protocol and disease duration. These data illustrate the distal to proximal progression of muscular involvement in DM, a pattern of progression well-recognized by the clinicians. The follow-up assessment of a large DM cohort in a longitudinal study will establish whether this quantitative protocol provides sensitive measures of the disease progression.     

An Electromyographic Study of the Hindlimb Locomotor Movements in the Acute Thalamic Rat

In this paper we have analysed the patterns of muscular activities that underlie hindlimb locomotor movements in the acute thalamic rat. Electromyographic activities of muscles representative of the functional muscle groups of the hindlimbs were recorded bipolarly during locomotion in acute thalamic rats. Locomotor movements occurred spontaneously, but could also be induced by electrical stimulation (0. 1 ms pulses; 30–70 Hz; 75–300 μA) of the lateral hypothalamic area. The two hindlimbs displayed a wide variety of coordination patterns during both types of locomotion. However, alternated coordination of the hindlimbs occurred more frequently during induced than during spontaneous locomotion. Correspondingly, the duration of the spontaneous step cycles had a tendency to be shorter than that of the evoked step cycles, although they had a quite similar range. The patterns of muscular activities within one hindlimb were similar during spontaneous and induced locomotion. During each step cycle, (i) the hip and ankle flexors usually displayed a single burst in alternation with that displayed by the hip, knee and ankle extensors, (ii) a double bursting pattern was sometimes observed in flexors during fast locomotor movements, (iii) within flexors and extensors, muscles were recruited sequentially, and (iv) the activation of other muscles (semitendinosus, rectus femoris, extensor and flexor digitorum longus) consisted of single or double bursting patterns.     

Congenital facioscapulohumeral muscular dystrophy associated with tongue atrophy and sensorineural hearing disturbance

An 18-year-old high-school boy presented facial muscle weakness since birth, and then developed wasting around the neck, shoulder girdle, upper arms, and thighs. He was born to un-consanguineous parents. His father had suffered from similar but milder muscle atrophy with predominance on the right side of the face and shoulder girdle since adolescence. His mother and his only sibling were clinically unaffected. Hearing disturbance was detected at the age of 6, and he also noted atrophy of the tongue and the bilateral thighs at the age of 10. The symptom progressed gradually. Neurological examination on admission revealed a well-developed boy (166 cm/60 kg) with a prominent facial diplegia with distinct proximal muscular atrophy of the extremities. Muscles of the tongue, neck, upper arms, shoulder and pelvic girdles, and hamstrings were markedly involved. The anterior tibial muscles were also affected, while the calf muscles were hypertrophic. High arched palate, X legs, mild lordoscoliosis were also noted. Serum CK was slightly increased (424 IU/l), and needle EMG in the extremities including the tongue revealed myopathic changes. Muscle CT demonstrated marked atrophy of the proximal muscles in the lower limbs and hypertrophy of the calf muscles. Audiogram showed bilateral sensorineural hearing disturbance. Muscle biopsy of the gastrocnemius showed myogenic as well as neurogenic changes consisting of atrophic and hypertrophic fibers with interstitial cellular infiltration, and type I fiber predominance. With these family history as well as clinical and laboratory examinations, this case could be diagnosed as "congenital facioscapulohumeral muscular dystrophy".(ABSTRACT TRUNCATED AT 250 WORDS)     

MRI findings in studies of distal myopathy with rimmed vacuoles

A case of distal myopathy with rimmed vacuoles was studied with MRI, which showed a characteristic distribution of the affected muscles. A 41-year-old man who presented a slowly progressive weakness in his lower legs starting 11 years previously was admitted to our hospital of further investigation. Neurological examinations showed muscular wasting and weakness in the neck flexors, the flexors of the forearm, the flexors and adductors of the thigh and the extensors of the lower legs. Needle electromyography showed a myopathic pattern. Muscle biopsy revealed a variation in fiber size, an increase in internal nuclei, fatty infiltration and scattered rimmed vacuoles in a histochemical study. Electron microscopy revealed that rimmed vacuoles contained numerous lamellar bodies and glycogen particles. T1 and T2 weighted MRI showed high signals in the m. adductor of thigh m. biceps femoris, m. semimembranosus, m. semitendinosus, m. tibialis anterior, m. tibialis posterior, m. extensor digitorum longus, m. extensor digitorum brevis, m. peroneus, and m. gastrocnemius. There were three merits for the application of MRI to distal myopathy, (1) easy detection of the affected muscles as fatty change is expressed with a high signal intensity by MRI, (2) no affection by the presence of bones in MRI, and (3) the possibility to have a transverse section and a sagittal and coronal section in MRI. In this case MRI was very useful to detect the affected muscles and to observe the progress.     

Computed tomography of the skeletal musculature in Becker-type muscular dystrophy and benign infantile spinal muscular atrophy

Results of computed tomographic (CT) examination of the skeletal musculature in 26 patients with Becker-type muscular dystrophy (BMD) and 12 patients with benign infantile spinal muscular atrophy (BISMA) are presented. Both disorders revealed strikingly different changes that may have important clinical significance. First, in BMD, CT abnormalities consisted of areas of decreased densities, at first appearing in part of the muscle, and then gradually spreading until the whole muscle was replaced by low-density tissue. In BISMA, however, low-density lesions were scattered throughout the muscle. Second, in BMD, some muscles were preferentially affected in an early stage of the disease and others were relatively spared, whereas in BISMA, the muscles were involved more or less simultaneously. Third, an enlargement in size (hypertrophy) was frequently observed in BMD in various muscles of the legs, whereas in BISMA, this phenomenon can sometimes be noted only in the gastrocnemius muscles.     

Fiber type and fiber size changes in selected thigh muscles six months after low thoracic spinal cord transection in adult cats: Exercise effects

The effects of low thoracic spinal cord transection on muscle weights, fiber type compositions and, fiber cross-sectional areas of selected hind limb muscles were studied. Adult cats were spinalized at T12 and maintained for approximately 6 months. Some spinalized cats were exercised on a treadmill 30 min/day, 5 days/week to determine the role of weight support in maintaining the muscle properties. Spinalization resulted in a significant decrease in the weights of most extensors, whereas the flexors or those that act as both flexors and extensors were maintained near control values. All muscles showed a significant increase in the percentage of fast-twitch fibers and decrease in slow oxidative fibers following spinalization. In addition, the predominant fast fiber type in each muscle tended to have the largest decrease in cross-sectional area in the spinalized cats. The atrophic and fiber type adaptations were less pronounced in the exercised cats. In contrast, the relative cross-sectional area of high oxidative fibers generally was similar among the three groups. These results demonstrated that the muscles below the level of the lesion became more "fast-like" histochemically following spinal cord transection, whereas the oxidative properties were relatively unaffected. Further, daily exercise involving weight support appeared to be an important deterrent to these atrophic responses, particularly in the muscles that normally have a postural function (i.e., the slow extensors).     

A novel MYH7 mutation with prominent paraspinal and proximal muscle involvement

Laing distal myopathy (LDM) is caused by mutations in the MYH7 gene, and known to have muscle weakness of distal limbs and neck flexors. Through whole exome sequencing, we identified a novel p.Ala1439Pro MYH7 mutation in a Korean LDM family. This missense mutation is located in more N-terminal than any reported rod domain LDM mutations. In the early stage of disease, the present patients showed similar clinical patterns to the previously described patients of LDM. However, in the later stage, fatty replacement and atrophy of paraspinal or proximal leg muscles was more severely marked than lower leg muscles, and asymmetric atrophies were observed in trapezius, subscapularis and adductor magnus muscles. Distal myopathy like LDM showed marked and predominant fatty infiltrations in paraspinal or proximal leg muscles with marked asymmetry. These observations expand the clinical spectrum of LDM with the MYH7 mutation.     

Pattern of paralysis and reconstructive operations after traumatic brachial plexus lesions

The aim of this study was to evaluate persistent patterns of paralysis after traumatic brachial plexus lesions. As a result, consecutive reconstructive operations according to our differential therapy concept are presented. Between 04/1994 and 12/2000 in 104 patients with brachial plexus palsy, the grade of muscle power of the affected upper extremities was evaluated prospectively. The neuromuscular patterns of defect showed, in most cases, insufficient muscle power grades of 0-2 for the deltoid muscle (90%), supraspinatus muscle (82%), infraspinatus muscle (93%), elbow flexors (67% to 77%), hand and finger extensors (69% to 71%), and the abductor and extensors of the thumb (67% to 70%). In corresponding frequency, the following operations were performed between 04/1994 and 06/2002: shoulder arthrodesis (n 26), trapezius transfer (n 80), rotation osteotomy of humerus (n 10), triceps to biceps transposition (n 11), transposition of forearm flexors or extensors/Steindler operation (n 12), latissimus transfer (n 7), pectoralis transfer (n 1), teres major transfer (n 1), transposition of forearm flexors to the tendons of extensor digitorum (n 19) and of the extensor pollicis longus (n 9), and wrist arthrodesis (n 5).On malfunction of muscles following brachial plexus lesions, taking into account the individual neuromuscular defect, passive joint function, and bony deformities, different procedures such as muscle transposition, arthrodesis, and corrective osteotomy can be performed to improve function of the upper extremity.     

Lipid storage myopathy in von Gierke's disease: a case report.

An 18-year-old girl with von Gierke's disease associated with a lipid storage myopathy is reported. The diagnosis of von Gierke's disease was made from decreased activity in glucose-6-phosphatase in the jejunal biopsy specimen. Neurologically she showed generalized hypotonia of the muscles, atrophy of bilateral proximal muscles of the lower extremities, weakness in neck flexors, deltoid and lumbar girdle muscles, and a positive Gower's sign. Muscle biopsy from flexor femoris muscle revealed fatty deposition in type 1 fibers and atrophy of type 2 fibers and the diagnosis of an accompanying lipid storage myopathy was made. This case also had a ventricular septal defect confirmed by right cardiac catheterization.     

Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study

Our aim was to determine isokinetic strength and degeneration of lower extremity muscles in patients with Myotonic Dystrophy (DM1). In 19 patients with DM1 and 19 matched controls, strength measured by isokinetic dynamometry was expressed as percentage of expected strength (ePct), adjusted for age, height, weight and gender. MRI of the hip, thigh and calf muscles were obtained. Fat fraction (FF), mean contractile cross-sectional area (cCSA) and specific strength (Nm/cm²) were calculated. Patients’ ankle plantar flexors, knee flexors and extensors had higher FF (Δ: 0.08 – 0.42) and lower cCSA (Δ: 3.2 –17.1 cm²) compared to controls (p ≤ 0.005). EPct (Δ: 19.5 – 41.6%) and specific strength (Δ: 0.27 – 0.96 Nm/cm²) were lower in the majority of patients muscle groups (p?0.05). Close correlations were found for patients when relating ePct to; FF for plantar flexors (R²=0.742, p<0.001) and knee extensors (R²=0.732, p<0.001), cCSA for plantar flexors (R²=0.696, p<0.001) and knee extensors (R²=0.633, p<0.001), and specific strength for dorsal flexors (ρ=0.855, p = 0.008). In conclusion, patients had weaker lower extremity muscles with higher FF, lower cCSA and specific strength compared to controls. Muscle degeneration determined by quantitative MRI strongly correlated to strength supporting its feasibility to quantify muscle dysfunction in DM1.     

Quantitative MR imaging of individual muscle involvement in facioscapulohumeral muscular dystrophy

The purpose of this study was to implement a quantitative MR imaging method for the determination of muscular and fat content in individual skeletal muscles of patients with facioscapulohumeral muscular dystrophy (FSHD).Turbo Inversion Recovery Magnitude (TIRM) and multiecho MR images were acquired from seven FSHD patients and healthy volunteers. Signal decay in the multiecho MR images was fitted to a biexponential function with fixed relaxation rates for muscle and fat tissue and used to calculate the degree of fatty infiltration in eight muscles in the lower leg.Considerable differences in fatty infiltration between different muscles were observed in FSHD patients, suggesting that this could be used as a biomarker for disease progression. TIRM imaging indicated an inflammatory component of the disease previously only observed in muscle biopsies. Typically, muscle involvement was non-uniform even within one muscle, indicating that MRI can be used as a valuable tool to study pathophysiology and therapy evaluation in FSHD.     

Muscular dystrophy with separate clinical phenotypes in a large family

This report describes a large consanguineous family with muscular dystrophy in 23 patients showing intrafamilial variation of clinical expression. One main variant appeared in the first decade with proximal muscle weakness progressing over the next 20 years to wheelchair confinement, and appeared compatible with classical limb-girdle muscular dystrophy. The other main variant showed onset of distal muscle weakness in lower limbs in the third or fourth decade, progressing very slowly without greater disability throughout the lifetime. Tibial muscle weakness and wasting were clinical landmarks in this variant, but computed tomography of skeletal muscle revealed focal areas of fatty degeneration also in truncal, pelvifemoral, and distal leg muscles in a way not previously reported in distal myopathy. The overall difference in clinical findings between these main variants would suggest 2 separate genetic entities, genealogical data makes a common genetic background possible.     

Effects of creatine supplementation on exercise performance and muscular strength in amyotrophic lateral sclerosis: preliminary results.

Creatine supplementation in humans has been reported to enhance power and strength both in normal subjects and in patients with various neuromuscular diseases. The purpose of this study was to examine the effects of supplementation on exercise performance and maximal voluntary isometric muscular contraction (MVIC) in Amyotrophic Lateral Sclerosis (ALS) patients.We report the results obtained in 28 patients with probable/definite ALS. In each patient we acquired the dynamometric measurement of MVIC in 10 muscle groups of upper and lower limbs and a measure of fatigue by means of an high-intensity intermittent protocol in elbow flexors and knee extensors muscles. All patients completed the protocols at the baseline and after supplementation of 20 g per day for 7 days and after supplementation of 3 g per day for 3 and 6 months. MVIC increased after 7 days of supplementation in 20 patients (70%) in knee extensors and in 15 (53%) of them also in elbow flexors. A statistically significant difference between pre and post-treatment mean values of MVIC was found both in elbow flexors (P<0.05) and knee extensors (p<0.04). The analysis of the slopes of fatigue test showed a statistically significant improvement after 7 days of supplementation in 11 patients (39%) in elbow flexors and in 9 patients (32%) also in knee extensors muscles. During the 6-month follow-up period all the examined parameters showed a linear progressive decline.In conclusion, our preliminary results have demonstrated that supplementation temporary increases maximal isometric power in ALS patients so it may be of potential benefit in situations such as high intensity activity and it can be proposed as a symptomatic treatment.