1/58. A congenital dermal sinus presenting the muscle fasciculation and hypertrophy.OBJECTIVE: To report unique and unknown clinical features of muscle fasciculation and muscle hypertrophy in a case of congenital dermal sinus. patients: A 16-year-old girl presented with continuous fasciculation, often cramp, and hypertrophy of the left calf muscle. The radiography showed spina bifida of L4, L5 and S1. MRI revealed dermal sinus tract from the skin dimple of the back to the dura mater, and connected to the intradural inclusion tumor. At surgery the inclusion tumor contained many short hairs, and the cauda equina were severely adherent. microdissection of the tumor and the adhesion was performed. At 2 years after surgery fasciculation decreased but continued; however, painful cramps of the calf muscle do not occur. CONCLUSIONS: Short hairs of dermoid and the adherence might be irritative to the cauda equina. The hyperactivity of the stimulated motor neuron may cause the muscle fasciculation leading to hypertrophy of the calf muscle.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/58. Pathophysiological significance of fasciculations in the early diagnosis of ALS.In amyotrophic lateral sclerosis (ALS), muscles with preserved strength can have fasciculation potentials (FPs) as the only abnormality. The FPs in strong muscles are predominantly simple and stable, and can often be recruited by a slight voluntary contraction. In weak and atrophic muscles, the FPs are generally complex and unstable, tend to have a slower firing rate and are not recruited by voluntary contraction. Macro-electromyography studies suggest that these FPs are part of a more complex motor unit, as opposed to simple FPs which can represent all the motor unit. FPs driven by transcranial magnetic stimulation (TMS) were observed in 13 ALS patients. TMS-driven fasciculations had a simple morphology and were stable. Complex potentials were never cortically driven. These observations strengthen the conviction that fasciculations in ALS can have different origins. Simple, stable FPs arise proximally and are probably related to excitotoxicity phenomena, while complex, unstable FPs are most likely to originate in distal axonal sprouts that are associated with the reinnervation process. Some ALS patients with fatigue and mild weakness can have profuse fasciculations at an early phase in the evolution of the disease. patients with denervation localized in one region and diffuse FPs should be strongly suspected of having ALS. With this strategy it should be possible to shorten the diagnosis time.- - - - - - - - - - ranking = 0.3keywords = muscle (Clic here for more details about this article) |
3/58. Fasciculations without fibrillations: the dilemma of early diagnosis.The challenge of identifying patients at the earliest stage of disease has highlighted the role of subtle markers of clinical pathology. Electrophysiological changes usually precede evidence of clinical weakness and have, therefore, been implicated. Specifically, the onset of fasciculations, particularly those widespread in distribution, in the absence of fibrillation potentials, have been suggested as an early indicator of motor neuron disease. Several cases will be presented here to highlight instances where diffuse fasciculations were not enough to accurately implicate the current diagnostic criteria for amyotrophic lateral sclerosis (ALS). In two instances, alternative diagnoses were eventually supported. If the purpose of early diagnosis is to lead to early treatment with experimental therapy, we must be certain that we study as homogenous a population of patients as possible. Fasciculations by themselves are not enough to implicate early diagnosis; disease progression is probably the single most important historical characteristic for diagnosis. Signs of active denervation (i.e. fibrillation potentials) are critical. Chronic weakness should be proportional to either atrophy or upper motor neuron signs in the affected muscle. This should be true for both early and late recognition of the disease.- - - - - - - - - - ranking = 0.44885692673381keywords = atrophy, muscle (Clic here for more details about this article) |
4/58. Progressive bulbar palsy: a case report diagnosed by lingual symptoms.The aim of this report is to show a case of Progressive Bulbar Palsy (PBP), diagnosed by oral medicine specialists, from oral symptoms of the disease. We have found no more than two published cases of PBP diagnosed by lingual alterations. We have followed the patient for almost four years, which is remarkable considering that the normal survival period for these patients is up to three years. We would like to emphasize the role of general dentists in the diagnosis of systemic conditions based on an oral examination that should include the oro-facial muscles.- - - - - - - - - - ranking = 0.1keywords = muscle (Clic here for more details about this article) |
5/58. Multifocal motor neuropathy and asymptomatic Hashimoto's thyroiditis: first report of an association.Motor neuropathy with multifocal conduction blocks represents a recently identified autoimmune disorder of the peripheral nerve myelin. association of motor neuropathies or neuronopathies with thyroid disorders, such as hyperthyroidism, hypothyroidism or thyroid neoplasms has been rarely described. We studied a 61-year-old man with a 2-year-history of slowly progressive weakness of the left limbs with atrophy and fasciculations. Nerve conduction velocity studies revealed multifocal motor conduction blocks. serum IgM titer of antibodies against GM1 was elevated (1:1280; n.v. up to 1:640). Thyroid studies were compatible with Hashimoto's thyroiditis. Therapy with high dose intravenous immunoglobulins was followed by a prompt clinical recovery. Then the disease assumed an intravenous immunoglobulins dependent course with a full clinical, but transient, recovery. This is the first observation of an association of multifocal motor neuropathy with high titers of GM1 and Hashimoto's thyroiditis and reinforces the multifocal motor neuropathy autoimmune origin as well as the repeated clinical recoveries after intravenous immunoglobulins. This case also suggests to deeply investigate the thyroid function in patients with multifocal motor neuropathy.- - - - - - - - - - ranking = 0.34885692673381keywords = atrophy (Clic here for more details about this article) |
6/58. Three-dimensional eye movement analysis of superior oblique myokymia.PURPOSE: To confirm the idea that sole contraction of the superior oblique muscle causes attacks of superior oblique myokymia (SOM). DESIGN: Observational case report. methods: A 43-year-old man presented with episodic monocular oscillatory eye movements. Three-dimensional eye movements were recorded using the magnetic search coil system and analyzed as rotation vector. The attacks of SOM consisted of intorsional, depressive, and abductive fast phases and their exponential decaying slow phases. RESULTS: Average direction of rotation axis for the fast phases while fixating straight ahead was 51.5 /- 5.4 degrees from depression axis in vertical-torsional plane, 78.7 /- 4.4 degrees from abduction axis in horizontal-torsional plane, and 76.3 /- 4.3 degrees from abduction axis in horizontal-vertical plane ( /-SD, n = 50). time constants of slow phases were less than 0.1 second. CONCLUSIONS: Quantitative three-dimensional analysis of SOM directly confirmed that the fast phases of SOM attacks were induced by the sole contraction of the superior oblique muscle.- - - - - - - - - - ranking = 0.2keywords = muscle (Clic here for more details about this article) |
7/58. Acute femoral neuropathy secondary to an iliacus muscle hematoma.We present a patient with a spontaneous iliacus muscle hematoma, appearing immediately after a minor physical maneuver, presenting with pain and femoral neuropathy initially evidenced by massive quadriceps muscle fasciculations. A magnetic resonance imaging (MRI) study of the pelvic area confirmed the diagnosis, showing a hematoma secondary to a partial muscle tear. The patient was managed conservatively, and the continuous muscle activity ceased in 3 days, with progressive improvement of the pain and weakness. The recovery was complete. femoral neuropathy is uncommon and usually due to compression from psoas muscle mass lesions of diverse nature, including hematomas. Usually subacute, femoral neuropathy may present acutely in cases of large or strategically placed compressive femoral nerve lesions, and may require surgical evacuation.The case presented herein is remarkable since the muscle hematoma appeared after a nonviolent maneuver, fasciculations were present at onset, and conservative management was sufficient for a full recovery.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
8/58. Normal conduction in pathways traversing an asymptomatic multiple sclerosis plaque.A 31-year-old woman developed right facial myokymia as the initial manifestation of multiple sclerosis (MS). An MRI scan revealed a focal signal abnormality confined to the left dorsolateral pontomedullary region. brain-stem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SEPs), and blink reflex (BR) failed to show a conduction abnormality through the left brain-stem lesion. Instead, BAEP and BR indicated a conduction defect in the right pons and EMG showed myokymic discharges in right facial muscles. Our findings provide rare documentation of normal conduction through a presumably asymptomatic MS plaque. The abnormal MRI signal likely represents tissue edema, rather than demyelination. This case demonstrates that physical findings in MS patients may correlate better with electrophysiological abnormalities than with MRI abnormalities.- - - - - - - - - - ranking = 0.1keywords = muscle (Clic here for more details about this article) |
9/58. Myokymic discharges: prompt cessation following nerve root decompression during spine surgery.In surgical cases during which spine nerve roots are at risk, we have found it useful to monitor EMG from the muscles supplied by those roots. Mechanical irritation of a root results in muscle activity, whose amplified signals can be broadcast over a loudspeaker, providing immediate feedback to the surgeon that the root is being irritated. We report here on a patient undergoing spinal canal decompression and fusion following a burst fracture of the L5 vertebral body sustained five days previously. EMG was continuously monitored from the tibialis anterior (TA) and medial gastrocnemius (MG) muscle groups bilaterally. During the period leading up to decompression, myokymic discharges from the left TA muscle were observed, whereas the other 3 muscles monitored did not show such activity. These semi-rhythmic and repetitive discharges from the left TA ceased immediately following surgical removal of a bone fragment compressing the left L5 nerve root. This indicates that the site of axonal irritation was the nerve root, and that myokymic discharges secondary to acute axonal compression can cease immediately upon nerve root decompression.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
10/58. Surgical management of myokymia of the superior oblique muscle.In the past 15 years, we have examined 20 patients in whom myokymia of the superior oblique muscle was diagnosed. Medical treatment (carbamazepine) failed in three patients, and one patient did not accept drug therapy. These four patients (20%) were operated on for persistent oscillopsia and diplopia. One patient underwent only a superior oblique muscle myotomy, but required an inferior oblique muscle myectomy six months later because of iatrogenic superior oblique muscle palsy. The other three patients underwent simultaneous superior oblique muscle myotomy and inferior oblique muscle myectomy. The symptoms resolved postoperatively in all four patients. Symptomatic patients with superior oblique muscle myokymia in whom medical treatment fails or is intolerable can benefit from surgical treatment consisting of combined superior oblique muscle/inferior oblique muscle myectomy.- - - - - - - - - - ranking = 1.3keywords = muscle (Clic here for more details about this article) |
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