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At least 1,243 records · Page 69Linked to original sources
PubMed1990

Acute alterations in murine neuromuscular transmission following exposure to a nonparalytic dose of dithiobiuret.

Treatment of rats for 5 to 6 days with dithiobiuret (DTB, 1 mg/kg/day, ip) causes a flaccid, ascending neuromuscular weakness which is associated with a decreased end-plate potential (EPP) amplitude, quantal content, miniature end-plate potential (MEPP) frequency, and prolongation of MEPP and EPP rise and decay times. Whereas small daily doses of DTB reliably cause this paresis, a single large dose, approximating the LD50, and far in excess of the cumulative dose given chronically to induce paralysis, causes no apparent muscle weakness. It was of interest to determine whether subtle changes in neuromuscular transmission are produced by DTB under dosing conditions in which gross muscle weakness is not apparent. As such the present study had two goals: first, to determine whether a single large dose of DTB (25 mg/kg, ip) altered neuromuscular transmission at times when the animal did not exhibit paresis; and second, to determine whether bath application of DTB, at concentrations approximating those in the animal following a single large dose, altered junctional transmission at early exposure times. EPPs and MEPPs were recorded, from hemidiaphragms taken 1, 4, 8, or 24 hr following treatment of rats with a single dose of DTB or vehicle or from untreated rats which were exposed to 200 microM or 1.85 mM DTB by bath application. One hour after a single large dose of DTB, EPP amplitude and MEPP frequency and amplitude were all decreased. Rise and decay times for MEPPs were prolonged in muscles taken 4 hr after treatment. By 4, 8, and 24 hr after treatment, EPP amplitude, MEPP amplitude, and MEPP frequency returned toward control levels. Bath application of DTB initially increased EPP amplitude, MEPP amplitude, and MEPP frequency; however, with continued exposure EPP amplitude decreased to below control levels. Block of EPPs occurred after approximately 10 or 37 min of exposure to 1.85 mM or 200 microM DTB, respectively. MEPP frequency also decreased with continued exposure to DTB, yet remained above control levels for the duration of DTB exposure. Bath application of DTB caused a slowing of decay times of MEPPs similar to that observed following in vivo exposure. These results demonstrate that a single large dose of DTB initially induces neuromuscular effects similar to those observed in rats paralyzed following chronic treatment with DTB but these effects, with the exception of effects on rise and decay times of synaptic potentials, tend to reverse by 24 hr following exposure.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1992

[A case of Hashimoto's thyroiditis associated with renal tubular acidosis, Sjögren syndrome and empty sella syndrome].

This report describes a 48-year old female patient with Hashimoto's thyroiditis, distal-type renal tubular acidosis (d-RTA), Sjögren syndrome (SjS), and empty sella syndrome (ESS). She has been receiving replacement of thyroxine for Hashimoto's thyroiditis since 1967. She felt muscle weakness and numbness in the extremities and was found to have low serum potassium (2.9 mEq/l) in 1987. Since then she has been administrated potassium chloride orally. She was admitted to our hospital because of recurrence of muscle weakness and numbness of the extremities in November 1990. Laboratory examination revealed that her serum levels of antimicrosomal antibody and anti-thyroglobulin antibody were highly positive (MCHA: x 2(10) x 100, and TGHA: x 100). Furthermore, she was revealed to have 1) d-RTA by oral tolerance tests with the administration of NH4Cl and NaHCO3, 2) SjS by Schirmer test and sialography, and 3) ESS by computed tomography and magnetic resonance imaging examinations of the pituitary. Association of Hashimoto's thyroiditis, d-RTA, SjS and ESS in this case may possibly be caused by common autoimmune mechanism.

Acidosis, Renal Tubular↗
PubMed1990

[Myopathy in acromegaly. Report of two cases].

Acromegaly is often associated with neuromuscular disorders. Most of them are caused by compression of nerves with hypertrophic bone and soft tissues or complications of diabetes mellitus. Myopathy has rarely been reported in the Japanese literature. We report two cases with myopathy out of 14 cases of acromegaly. Case 1 is a 62-year-old woman who developed muscle weakness and atrophy in the shoulder girdle, pelvic girdle and femoral regions after a 10-year history of acromegaly. She showed positive Gowers' sign and normal DTRs. Basal growth hormone (GH) level in plasma was 1076 ng/ml. Electromyograms (EMG) obtained from the deltoid and rectus femoris muscles revealed typical myopathic abnormalities; an excess of small-amplitude, short-duration, polyphasic motor unit potentials. Histological examinations of the rectus femoris muscle showed diffuse atrophy of both type I and type II fibers. She also had bilateral carpal tunnel syndrome and bilateral tarsal tunnel syndrome, which were confirmed by nerve conduction studies of median nerves and posterior tibial nerves. A cranial computed tomography (CT) scan demonstrated sellar mass with suprasellar extension. She underwent transsphenoidal adenomectomy and radiation therapy. GH level lowered to 29 ng/ml, however, myopathy remained unchanged for 3 years after the surgery. Case 2 is a 38-year-old woman who had undergone partial removal of a pituitary adenoma 9 years after the onset of acromegaly. Basal GH level in plasma before the surgery had been 1694 ng/ml and was still high after the surgery (100-505 ng/ml). The patient developed proximal muscle weakness and atrophy 4 years after the surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Acromegaly↗
PubMed2001

Symptoms of thoracolumbar junction disc herniation.

STUDY DESIGN: A retrospective clinical review of patients with thoracolumbar junction disc herniation. OBJECTIVES: To evaluate the clinical features of thoracolumbar junction disc herniation and to prepare a chart for the level diagnosis in the neurologic findings and symptoms. SUMMARY OF BACKGROUND DATA: Thoracolumbar junction disc herniations show a variety of signs and symptoms because of the complexity of the upper and lower neurons of the spinal cord, cauda equina, and nerve roots. Furthermore, much is still unknown about thoracolumbar junction disc herniations because of their rare frequency. METHODS: The clinical features of 26 patients who had undergone operations for single disc herniations at T10-T11 through L2-L3 were investigated. Affected levels were as follows: 2 patients with disc herniation at T10-T11 disc, 4 patients at T11-T12, 3 patients at T12-L1, 6 patients at L1-L2, and 11 patients at L2-L3. The level of disc space of interest was confirmed with whole-spine plain roentgenograms. The caudal end of the cord was judged by magnetic resonance imaging and computed tomographic myelogram. RESULTS: Two patients with T10-T11 disc herniation showed moderate lower extremity weakness, increased patellar tendon reflex, and sensory disturbance of the entire lower extremities. Three of four patients with T11-T12 disc herniation experienced lower extremity weakness, and three patients had accentuated patellar tendon reflex. Sensory disturbance was observed in the anterolateral aspect of the thigh in one patient and on the entire leg in three patients. Bowel and bladder dysfunction was noted in three patients. In the T12-L1 disc herniation group (n = 3), muscle weakness and atrophy below the leg were advanced, and bowel and bladder dysfunction were also noted. Two of these three patients had bilateral drop foot, and one patient had unilateral drop foot; sensory disturbance was noted in the sole or foot and around the circumference of the anus, and the patellar tendon reflex and Achilles tendon reflex were absent. All six patients with L1-L2 disc herniation showed severe thigh pain and sensory disturbance at the anterior aspect or lateral aspect of the thigh. On the other hand, there were no clear signs of lower extremity weakness, muscle atrophy, deep tendon reflex, or bowel and bladder dysfunction in these patients. In the L2-L3 disc herniation group (n = 11), all patients had severe thigh pain and sensory disturbance of the anterior aspect or the lateral aspect of the thigh. Weakness in the quadriceps was noted in five patients and weakness in the tibialis anterior in two patients. Decreased or absence of patellar tendon reflex was observed in nine patients. Five patients had positive straight leg raising test results, and eight patients showed positive femoral nerve stretch test results. CONCLUSION: Among thoracolumbar junction disc herniations, T10-T11 and T11-T12 disc herniations were considered upper neuron disorders, T12-L1 disc herniations were considered lower neuron disorders, L1-L2 disc herniations were considered mild disorders of the cauda equina and radiculopathy, and L2-L3 disc herniations were considered radiculopathy. These findings had relatively distinct differences among herniated disc levels.

Adult↗
PubMed1978

Skeletal muscle characteristics and carbohydrate metabolism after glucose loading in hypokalaemic periodic paralysis.

A prolonged glucose load was administered to four patients with hypokalaemic periodic paralysis and four healthy control sujbects. Muscle ATP and CP concentrations as well as lactate dehydrogenase, hexokinase and phosphorylase activities were similar in those two groups, but succinate dehydrogenase was approximately 50% higher in the control muscles. Muscles fibre composition was almost identical in the two groups, whereas patients had a higher degree of capillarization. Complete muscle weakness was produced in all patients, accompanied by hypokalaemia. Glucose loading resulted in elevated insulin levels and a minor rise in blood glucose level was seen in the patients compared to the control subjects. Glucose loading decreased hexokinase activity in controls, but increased this in the patients. At similar times, muscle and blood lactate levels and blood pyruvate values were generally higher in the patients over the course of the experiment. Initial glycogen concentrations were higher in patients, but glucose loading did not result in greatly increased glycogen values. These data suggest that patients with hypokalaemic periodic paralysis have an enhanced metabolism of carbohydrates and that insulin seems to be an important factor leading to the onset of muscle weakness.

Adenosine Triphosphate↗
PubMed2006

Joint-angle-dependent neuromuscular dysfunctions at the wrist in persons after stroke.

OBJECTIVE: To evaluate the joint-angle-dependent neuromuscular functions at the affected wrist in hemiplegic subjects after stroke while doing isometric maximal voluntary wrist flexion and extension across different wrist angles. DESIGN: We investigated torques during isometric maximal voluntary wrist flexions and extensions at 8 different wrist angles, ranging from -45 degrees to 60 degrees. We used the associated electromyographic activities of 2 agonist and antagonist muscle pairs related to wrist and elbow joints for the analysis of muscular coactivations. We compared the data obtained from poststroke subjects' affected and unaffected sides. SETTING: A research laboratory in a rehabilitation center. PARTICIPANTS: Eleven subjects with hemiplegia after stroke with passive range of motion (ROM) in the wrist from -45 degrees to 60 degrees. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Directly measured torques, torques after normalization during maximal isometric wrist contractions, and normalized moving average electromyographic signals of each muscle at the tested positions. RESULTS: The measured torques of the affected wrists were significantly lower than those of the unaffected wrists at all tested angles during wrist flexion and extension (P<.05). The angle-dependent patterns of the normalized torque across the tested wrist angles varied from those of the unaffected wrists (2-way analysis of variance, P<.05). There were decreases in normalized torques during both flexion and extension at the extended positions in the affected group (P<.05). Abnormal cocontractions were found in agonist and antagonist muscle pairs related to wrist and elbow joints, and between the elbow flexor and wrist extensor when subjects did the wrist contractions on the paretic side, especially at the wrist extended positions. CONCLUSIONS: Wrist muscle weakness was distributed unevenly across the selected wrist ROM on the affected side, as represented by the varied patterns of the normalized torque-angle relationship, compared with the unaffected wrists. There were reductions in the selective control of muscle coactivating synergies both single-jointly and cross-jointly in the impaired nervous system during wrist contractions; the extent of these reductions was also related to the wrist angle configuration.

Adult↗
PubMed1998

[Ataxic form of Guillain-Barré syndrome associated with acute hepatitis A--a case report].

A 46-year-old male developed a fever of 38 degrees C and general fatigue, and two days later dysesthesia of limbs and muscle weakness of upper extremities appeared. He had difficulty in walking because of truncal and limb titubation and was admitted to our hospital. On admission, pin-prick and soft-touch sensation was reduced in the distal portion of all four extremities, and mild muscle weakness of limbs was noted. He also presented marked disturbance of vibratory and position sense, and had sensory ataxia. All deep tendon reflexes were absent. Moderate liver dysfunction was noted and serum IgM-HA antibody was positive. CSF total protein was elevated to 123 mg/dl on admission. Motor conduction velocity was mildly delayed in the lower extremities, and sensory conduction velocity was mildly delayed in the ulnar nerves and the left sural nerve. The serum of the patient had low titer of IgG anti-sulfated glucuronyl paragloboside (SGPG) antibody. The biopsy specimen from his right quadriceps femoris muscle showed subsarcolemmal aggregates of abnormally enlarged mitochondria with paracrystalline inclusions by electron microscopy. After admission neurological symptoms worsened. Severe diturbance of deep sensation and severe sensory ataxia persisted for a month and slowly improved thereafter. CSF total protein was elevated up to 760 mg/dl. He was treated with plasmapheresis of 12 times and was discharged on the 176th day after the onset, but he could not still walk by himself at that time. Diturbance of deep sensation still remained for one and a half years after the onset. Guillain-Barré syndrome (GBS) associated with acute hepatitis A is rare. In addition, there have been no previous reports describing abnormal mitochondrial changes of the skeletal muscle in GBS.

Acute Disease↗
PubMedDate not supplied

[Infant botulism and sudden infant death syndrome].

Infant botulism represents a distinct entity of botulism. Ingestion of the ubiquitously present spores of Clostridium botulinum leads to germination of the organism and neurotoxin production in the infant intestine. Symptoms typically develop gradually in contrast to classical food botulism in which an acute onset of symptoms shortly after the ingestion of preformed toxin in a food is characteristic. Microbiologically, the diagnosis is established by identification of Clostridium botulinum organism and toxin in stool specimen. However, positive results in these tests provide only indirect evidence for the clinical relevance of the neurotoxin since asymptomatic carriers have been found. The toxin irreversibly blocks the release of acetylcholin from the motoric end plate which results in muscle weakness and paralysis. Depending on the amount of toxin produced, infant botulism exhibits a broad clinical spectrum ranging from oligosymptomatic forms to a fulminant course with acute respiratory failure within hours leading to sudden death. Unrecognized mild forms or beginning muscle weakness can be a co-factor for other risk factors of sudden infant death (SIDS). In studies analyzing infants who died from SIDS, botulism bacteria or toxin were found in up to 20 % of cases. Infant botulism therefore represents an important differential diagnosis of unexplained and inconclusive muscular hypotonia in the first year of life.

Acetylcholine↗
PubMed2006

Pure myopathy associated with a novel mitochondrial tRNA gene mutation.

The authors describe a 47-year-old man who presented with proximal muscle weakness, myalgia, elevated creatine kinase, and features of a pure myopathic syndrome in whom they have identified a novel mutation in the mitochondrial tRNA(Ala) gene. This 5591G>A transition is heteroplasmic, segregates with cytochrome c oxidase deficiency in single muscle fibers, and fulfills recognized criteria for pathogenicity. This case exemplifies the wide-ranging clinical spectrum of mitochondrial disease presentations.

Creatine Kinase↗
PubMedDate not supplied

Acromegaly as an endocrine form of myopathy: case report and review of literature.

OBJECTIVE: To describe a case of muscle weakness in a patient with acromegaly and to review the pathophysiologic features of this disorder. METHODS: We present the clinical, laboratory, electromyographic, and muscle biopsy findings in our patient and review related reports in the literature. RESULTS: A 58-year-old woman with acromegaly presented with complaints of bilateral hip pain, weakness, and instability 8 months after transsphenoidal resection of a growth hormone (GH)-secreting pituitary macroadenoma. She had biochemically normal thyroid and adrenal function and no evidence of any neuropathy, inflammatory myopathy, or rheumatologic disorder to explain her symptoms. Investigations revealed increased levels of GH, insulin-like growth factor-I, serum creatine kinase (CK), and the MB fraction of CK, normal results of nerve conduction studies, and nonspecific findings on electromyography and muscle biopsy. A review of the literature revealed that although muscle weakness is a well-recognized feature of acromegaly, only a few cases similar to ours have been reported since acromegaly was first described in the late 1800s. Little is known about the natural history, best diagnostic approach, and optimal therapy for this debilitating complication. CONCLUSION: Muscle weakness in acromegaly is common and may result from a combination of the direct effect of GH excess on muscle and other metabolic derangements (hypothyroidism, hypoadrenalism, or diabetes). Mechanical factors may also contribute, such as joint laxity in conjunction with hypermobility. Affected patients may benefit from a reduction in GH levels and physiotherapy for adaptive training. Persistently increased serum CK levels in a patient with diabetes, for whom no other cause is found, should prompt an investigation for acromegaly. More research into this aspect of acromegaly is needed for enhancement of our understanding of, and therapy for, this debilitating condition.

Acromegaly↗
PubMed2001

[Myotonic dystrophy (DM/Curschmann-Steinert disease) and proximal myotonic myopathy (PROMM/Ricker syndrome). Myotonic muscle diseases with multisystemic manifestations].

Myotonic disorders are characterised by slowed muscle relaxation and myotonic discharges in the electromyogram. "Pure" myotonic disorders affect only muscle and can be separated into ion channel disorders affecting either the chloride channel (myotonia congenita Thomsen or myotonia congenita Becker) or those affecting the sodium channel (paramyotonia, hyperkalemic periodic paralysis, and myotonia fluctuans). The genetic defects in these disorders are point mutations or deletions within the respective channel genes. A second group of myotonias consists of multisystem disorders with muscle weakness and atrophy plus extramuscular symptoms and signs including cardiac arrhythmias, cataracts, hypogonadism, and pain. Classic myotonic dystrophy (Steinert's disease or DM 1), and proximal myotonic myopathy (PROMM or Ricker's syndrome) are the major syndromes. PROMM is characterised by predominantly proximal muscle weakness and myalgias. Similarly to DM 1, anticipation also occurs in PROMM, but the disease course is usually milder. Steinert's disease belongs to the group of trinucleotide repeat-associated disorders. The DM 1 mutation is an unstable CTG trinucleotide repeat expansion on chromosome 19q13.3 which is diagnostic for the disease. A number of families with PROMM have been linked to a gene locus on chromosome 3q, but the mutation is still unknown. Therefore, direct molecular genetic testing for PROMM is not yet possible.

Biopsy↗
PubMed1990

[Cardiomyopathy in Becker muscular dystrophy].

In 6 patients with dystrophin-verified Becker muscular dystrophy (BMD), 3 patients had dilated cardiomyopathy (DCM group). The other 3 patients (non-DCM group) also had ECG abnormalities including incomplete right bundle branch block, left ventricular enlargement and intraventricular conduction defect. Between DCM and non-DCM group, there was no prominent difference in ages at onset, mean duration and severity of muscular weakness. Serum CK levels, and molecular weight and amount of dystrophin also showed no significant difference between two groups. On reviewing 14 BMD patients, including 3 present patients with cardiomyopathy, the cardiac symptoms appeared from 4 to 41 years, averaging 17.1 years of age. The mean duration of muscle symptoms was 9 years, ranging from 0 to 33 years. There was no correlation between severity of muscle weakness and cardiomyopathy. Six patients died of heart failure and 3 received cardiac transplantation. Thus there was no characteristic clinical feature in BMD patients with cardiomyopathy except for very poor prognosis. Since the myocardial involvement is not related with clinical severity and duration of the disease, careful observation for cardiac function should be carried out in all BMD patients even in the early stage of muscle weakness.

Adolescent↗
PubMed2005

Umbilical movement after spinal cord injury.

BACKGROUND/OBJECTIVE: Charles Beevor first discussed the movement of the umbilicus seen upon abdominal muscle contraction in neuromuscular diseases and spinal cord injury (SCI) over a century ago. Since then, other examples of this phenomenon have been reported in the literature. METHODS: This case review study documents movements of the umbilicus caused by abdominal muscle weakness after SCI. RESULTS: SCI results in characteristic weakness of the abdominal muscles resulting in predictable movement of the umbilicus upon abdominal muscle contraction. CONCLUSIONS: Observation of umbilical movement with abdominal muscle contraction after SCI gives further confirmatory information about neurological level and type of spinal cord lesion. Further study should include information about the effect of abdominal weakness on motor and respiratory function.

Abdominal Muscles↗
PubMed2003

Benign calf amyotrophy: clinicopathologic study of 8 patients.

BACKGROUND: The benign focal amyotrophy disorders have been described since 1959 for the upper limbs and since 1981 for the lower limbs. The clinicopathologic features have pointed to a restricted and self-limiting form of motor neuron disease. OBJECTIVE: To describe the clinical, electromyographic, and muscle histopathologic features in 8 patients with benign calf amyotrophy. DESIGN: Retrospective review of patient charts, electromyograms, and muscle histopathology. PATIENTS AND RESULTS: Eight patients, aged 37 to 88 years, developed insidiously progressive calf muscle weakness and wasting during 1 to 5 years. The gastrocnemius weakness and wasting were bilateral in 4 patients. Initial progression of symptoms was followed by disease stabilization. None had a history of poliomyelitis or family history of neuromuscular disease. Creatine kinase values were mildly elevated in 5 patients. The electromyographic and muscle histopathologic findings were consistent with a chronic neuropathic disorder. Despite the restricted calf muscle involvement clinically, the electromyographic abnormalities suggested more diffuse lower limb involvement. Further studies, including DNA tests and muscle-based protein studies, excluded several types of inherited neuromuscular disorders. CONCLUSIONS: Benign calf amyotrophy is a variant of the benign focal amyotrophy disorders. The etiology for these disorders is unknown. Studies to exclude other causes of calf amyotrophy and careful follow-up examinations to document disease stabilization are necessary to diagnose this uncommon disorder.

Adult↗
PubMed1997

Myotonic dystrophy in pregnancy: a report of two cases within one family.

myotonic dystrophy, also called the Curschmann-Steinert syndrome, is an autosomal dominant inherited neuromuscular disorder characterized by progressive muscular dystrophy, muscle weakness and myotonia, which can affect both mother and child. Complications may arise during pregnancy, delivery, including anaesthetic problems, and in the neonatal period. During pregnancy hydramnion can be a first sign of the disease leading to premature labor and also muscle weakness and myotonia can aggravate complicating the course of delivery. The affected neonate may display severe hypotonia, facial diplegia and respiratory distress. The clinical diagnosis can be confirmed by direct DNA analysis in serum and in chorionvillus biopsy material. In this case report two sisters with myotonic dystrophy are described, their pregnancies, deliveries and the outcome of their affected babies.

Adult↗
PubMed2005

[Systemic lupus erythematosus and dermatomyositis--case report].

The association of systemic lupus erythematosus (SLE) with idiopathic polymyositis or dermatomyositis is reported to occur in the range of 4-16%. Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously. This case report concerns a 36-year-old female patient suffering from Raynaud's phenomenon, polyarthralgia in the small joints of the hands, and skin changes compatible with Gotron's indications. Symmetric proximal muscle weakness of the extremities, fever of up to 40 degrees C, heliotrope rashes with erythematous changes in the face, upper arms, and posterior shoulders occurred subsequently. Laboratory analyses revealed increased acute phase reactants, hypochromic anaemia, lymphopenia, and increased levels of all muscle enzymes. Immunoserology demonstrated positive ANA, anti-Sm, and anticardiolipin antibodies (aCL), while anti dsDNA, anti Ro, anti La, and anti Jo-1 antibodies proved negative. Hypocomplementaemia and elevated levels of immune complexes were also detected. Pathologic sediment and proteinuria were revealed via urine analyses, while a kidney biopsy confirmed lupus nephritis (type IVa according to the World Health Organisation classification). Biopsy of erythematous changes of the posterior shoulder demonstrated leukocytoclastic vasculitis. Electromyography of the lower extremities established myopathic changes. Inflammation of the muscles was confirmed via magnetic resonance imaging. The patient was categorised as having two separate coexistent diseases--SLE and dermatomyositis. Both the classification criteria of the American College of Rheumatology for SLE and the diagnostic criteria for dermatomyositis, proposed by Bohon and Peter, were fulfilled simultaneously. Treatment commenced with pulses of methylprednisolone and continued with oral therapy, including Resochin. Pulses of intravenous cyclophosphamide were also administered. After six weeks of therapy, biohumoral remission of both diseases was achieved, while complete recovery from muscle weakness was accomplished after four months.

Adult↗
PubMed2002

Amyotrophic lateral sclerosis: evaluation and treatment of respiratory impairment.

Patients with amyotrophic lateral sclerosis (ALS) invariably develop respiratory muscle weakness and most die from pulmonary complications. There are numerous tests available to evaluate respiratory status in ALS and it is important to understand their various advantages and limitations. Forced vital capacity (FVC) is commonly used but can remain normal despite substantial inspiratory muscle weakness. Maximal pressures measured at the mouth are useful for excluding weakness if they are normal but are difficult to interpret if abnormal. Invasive testing, such as measurement of transdiaphragmatic pressure, provides an accurate measure of inspiratory strength but is not readily available and is not practical for serial measures. There are supportive respiratory techniques that have been shown to benefit patients with ALS. Clinicians should be familiar with these interventions, including mechanically assisted coughing, non-invasive ventilation and tracheostomy with mechanical ventilation. Observational studies have demonstrated improved survival and quality of life with noninvasive ventilation. Tracheostomy with long-term mechanical ventilation is not frequently used but can be an important component of care for ALS. This review describes an approach to respiratory evaluation and care of patients with ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1998

[Dramatic improvement with immunoabsorption therapy in a 7-year-old girl with severe Guillain-Barré syndrome after unsuccessful gammaglobulin therapy].

We report a 7-year-old girl with severe Guillain-Barré syndrome (GBS) who showed dramatic improvement after immunoabsorption therapy. She had progressive muscle weakness with resultant respiratory failure. On the 7th day of the illness, she was nearly quadriplegic and dependent on mechanical ventilation. In addition, she had autonomic manifestations, areflexia, abolished or delayed peripheral nerve conduction, and increased CSF protein. Administration of a high dose of gammaglobulin failed to improve the symptoms. Immunoabsorption therapy using a tryptophan column was performed 9 times during 17 days. After 5 times of immunoabsorption therapy, the muscle weakness improved dramatically; she was extubated 16 days after the institution of the therapy and walked without assistance on the 41st day. She regained normal muscle power without any sequelae. The immunoabsorption therapy caused hemolysis and decrease of serum fibrinogen, but no serious complications. The clinical course of this patient suggests the efficacy of immunoabsorption therapy in GBS.

Child↗