1/446. Cervical cord tethering mimicking focal muscular atrophy.spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. skin lesion at the nuchal area should be carefully looked for.- - - - - - - - - - ranking = 1keywords = muscular atrophy, atrophy, muscle (Clic here for more details about this article) |
2/446. MRI findings of benign monomelic amyotrophy of lower limb.We report here magnetic resonance imaging (MRI) findings of two patients with benign monomelic amyotrophy of lower limb. Both subjects showed unilateral amyotrophy of the lower limb with a benign clinical course, and the affected muscles demonstrated neurogenic changes. On T1- and T2-weighted MRI, marked atrophy and increased signal intensity were found mainly in gastrocnemius and soleus muscles. Moreover, MRI examination also revealed that thigh muscles including semitendinosus, semimembranosus, and vastus intermedius and lateralis muscles were involved in one of the patients. We concluded that muscle MRI is very useful for detecting affected muscles, especially deep skeletal muscles in patients with benign monomelic amyotrophy of lower limb.- - - - - - - - - - ranking = 0.0041375902290615keywords = atrophy, muscle (Clic here for more details about this article) |
3/446. Atraumatic palmar midcarpal dislocation in a skeletally immature adolescent with hemiatrophy.We report a late presentation of a palmar midcarpal dislocation in an adolescent female with open growth plates who had no history of antecedent wrist injury. Midcarpal arthrodesis improved function and eliminated progressive pain.- - - - - - - - - - ranking = 0.016154739658195keywords = atrophy (Clic here for more details about this article) |
4/446. motor neuron disease with predominantly upper extremity involvement: a clinicopathological study.We report two autopsy cases of motor neuron disease (MND) patients with an unusual type of muscular atrophy predominantly affecting the shoulder girdle and the upper extremities with proximal dominance. Both patients are considered to be clinically categorized into the El Escorial suspected form of amyotrophic lateral sclerosis (ALS). At autopsy, they showed marked loss of spinal anterior horn cells accompanied by astrogliosis positively immunostained with anti-glial fibrillary acidic protein antibody at the cervical level. At the lumbosacral level, anterior horn neurons were relatively well preserved and Bunina bodies, ubiquitin-positive skein-like inclusions and Lewy body-like inclusions were observed in the remaining neurons. In one patient, brain stem motor neurons (nerves V, VII, XII) and motor cortex, including Betz cells, were also affected and the corticospinal tracts were degenerated at the level of the thoracic and lumbar spinal cord. Pathological findings of this patient are consistent with those of ALS. In the other patient, the motor cortex, brain stem motor nuclei and the corticospinal tracts were well preserved, which is pathologically compatible with progressive spinal muscular atrophy. These patients with such a peculiar pattern of progressive muscular atrophy should be placed in a subgroup of ALS.- - - - - - - - - - ranking = 0.59999152240161keywords = muscular atrophy, atrophy (Clic here for more details about this article) |
5/446. Acute quadriplegic myopathy in a 17-month-old boy.Acute quadriplegic myopathy is a rare condition associated with the use of nondepolarizing muscle-blocking agents and corticosteroids in the course of severe systemic illness. A 17-month-old boy underwent liver transplantation for fulminant hepatitis. He was intubated for 24 days and treated with vecuronium bromide and high-dose methylprednisolone. The child was weaned from the ventilator and presented extreme weakness in the upper limbs and total paralysis of the lower limbs. serum creatine kinase level was normal and electromyography showed myopathic abnormalities. Muscle biopsy showed severe type-1 fiber atrophy and selective loss of myosin thick filaments was seen on electron microscopy. Scattered regenerating fetal myosin-positive fibers were present, mu calpain was absent, while m calpain was diffusely expressed. Physical therapy was immediately started and the child recovered even though corticosteroids were not discontinued. The pathogenesis of acute quadriplegic myopathy is still unknown. We suggest that it could be due to abnormal protein turnover in the muscle. Several independent factors, such as corticosteroid treatment, immobilization, or cytokines, could take part in a cascade of events that leads to an excessive yet selective degradation of proteins involving myosin thick filaments and possibly components of sarcolemma, causing muscle inexcitability.- - - - - - - - - - ranking = 0.0040810729064828keywords = atrophy, muscle (Clic here for more details about this article) |
6/446. Parkinsonism, dystonia, and hemiatrophy.Hemiatrophy has been reported in association with a variety of neurologic conditions, including parkinsonism. patients with the hemiparkinson-hemiatrophy syndrome (HP-HA) have asymmetric parkinsonism with limb atrophy on the more affected side. Several authors have suggested that asymmetric brain damage early in life results in both atrophy and parkinsonism. Dopa-responsive dystonia (DRD) is a disease in which a deficiency of tetrahydrobiopterin, or, less commonly, of tyrosine hydroxylase, results in levodopa-responsive dystonia with parkinson features in children. We have recently identified four patients with DRD who had asymmetric dystonia and limb atrophy on the more affected side. Based on these patients, we suggest that a deficiency of the nigrostriatal dopamine system may, by itself, be sufficient to cause body atrophy and may underlie the limb atrophy in both DRD and HP-HA.- - - - - - - - - - ranking = 0.044425534060036keywords = atrophy (Clic here for more details about this article) |
7/446. Unilateral pallidal stimulation in cervical dystonia.Cervical dystonia (spasmodic torticollis) is a focal dystonia of the cervical region. Various treatment modalities have been performed with variable success rates. We present a 42-year-old woman complaining of involuntary head rotation for the last 3 years. Different medical treatments had been used for 3 years. Botulinum toxin injections resulted in temporary and moderate improvement for periods of 3-4 months. Pallidal stimulation was performed using a quadripolar electrode and a battery-operated programmable pulse generator. We conclude that a unilateral pallidal lesion or stimulation is an effective method of treatment in focal dystonia. The target must be the pallidum contralateral to the contracted sternocloidomastoid muscle. deep brain stimulation is superior to lesioning because of the capability of manipulating the stimulation parameters which can modify the pallidotomy effect.- - - - - - - - - - ranking = 1.4129330644686E-5keywords = muscle (Clic here for more details about this article) |
8/446. ulnar nerve injuries of the hand producing intrinsic muscle denervation on magnetic resonance imaging.Muscle and nerve injuries in the hand may be difficult to detect and diagnose clinically. Two cases are reported in which magnetic resonance imaging showed ulnar nerve injury and intrinsic hand muscle denervation. The clinical, anatomical and radiological features of injury to the deep motor branch of the ulnar nerve and associated muscle denervation are discussed and illustrated.- - - - - - - - - - ranking = 8.4775983868116E-5keywords = muscle (Clic here for more details about this article) |
9/446. Complete rupture of the distal semimembranosus tendon with secondary hamstring muscles atrophy: MR findings in two cases.Complete rupture of the hamstring muscles is a rare injury. The proximal musculo-tendinous junction is the most frequent site of rupture. We present two cases of complete rupture of the distal semimembranosus tendon, which clinically presented as soft-tissue masses. MR imaging permitted the correct diagnosis. There has been only one other such case reported.- - - - - - - - - - ranking = 0.016225386311418keywords = atrophy, muscle (Clic here for more details about this article) |
10/446. Isolated hypoglossal nerve palsy due to an anomalous vertebral artery course: report of two cases.The authors report two patients with isolated unilateral tongue atrophy. magnetic resonance imaging (MRI) of the brain stem and angio-MRI demonstrated a dolichovertebral artery with an abnormal course compressing the medulla oblongata at the emergence of the hypoglossal rootlets. The semeiological observation of a sectorial and not uniform distribution of atrophy in the half-affected tongue is discussed in relation to the lesional site.- - - - - - - - - - ranking = 0.0080773698290975keywords = atrophy (Clic here for more details about this article) |
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