1/49. The guillain-barre syndrome following dengue fever.A 44 year old female presented with fever, muscle aches, rash and a low platelet count. IgM antibody to dengue virus was positive. Two weeks later she developed a flaccid areflexic quadriparesis. Nerve conduction studies showed a predominantly demyelitinating sensory motor polyneuropathy consistent with guillain-barre syndrome. Despite the relatively common occurrence of dengue fever, an associated polyradiculoneuropathy is distinctly uncommon.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/49. Postural and action myoclonus in patients with parkinsonian type multiple system atrophy.patients with a parkinsonian syndrome and features of multisystem atrophy (pMSA) may exhibit abnormal movements of the hands and fingers, which are reported in the literature either as "jerky" tremor or myoclonus. We studied clinically and electrophysiologically these movements in 11 consecutive patients with pMSA. No abnormal movements were observed when the patients were at complete rest, except for a characteristic parkinsonian "pill-rolling" tremor in one patient. Abnormal small-amplitude, nonrhythmic movements involving just one or a few fingers, or more rarely the whole hand, were observed in nine patients when holding a posture or at the beginning of an action. Accelerometric recordings showed small-amplitude irregular oscillations which, contrary to those of patients with tremor, had no predominant peak in the Fast Fourier frequency spectrum analysis. Electromyographic recordings in the forearm and hand muscles showed brief jerks of less than 100 ms duration which were synchronous in antagonist muscles of the forearm and alternated with brief periods of silence. Electrical stimulation of the digital nerves evoked consistent reflex responses in the wrist flexor and extensor muscles at a latency of 55.3 /-4.1 ms (range, 50-63 ms). Routine electroencephalographic (EEG) and somatosensory evoked potentials to median nerve stimulation were normal. back-averaging of the EEG activity time-locked to the jerks was performed in two patients with no evidence of abnormal cortical activity. Two patients had episodes of transient respiratory failure related to pneumonia. This caused a long-lasting enhancement of the abnormal hand and finger movements, which became larger and more widespread, with features of posthypoxic myoclonus. We conclude that the abnormal hand and finger movements of patients with pMSA are a form of postural and action myoclonus, and can be described as mini-polymyoclonus.- - - - - - - - - - ranking = 3keywords = muscle (Clic here for more details about this article) |
3/49. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.- - - - - - - - - - ranking = 4keywords = muscle (Clic here for more details about this article) |
4/49. An unusual case of hyperekplexia.Hyperekplexia is a rare paroxysmal disorder characterized by exaggerated startle response, hypertonia during infancy and a transient increase in tone following startle attacks. We report an unusual case of hyperekplexia in a young man. In addition to common symptoms of the condition, we found generalized spasticity persisting beyond infancy, and facial and skeletal dysmorphism. Because of an unsteady gait with frequent falls and raised serum creatine kinase levels, a congenital myopathy had been suspected in the past and an abnormal muscle biopsy had been documented. We diagnosed hyperekplexia at the age of 21 years on clinical grounds and following the response to pharmacological treatment. A mutation in the alpha1 subunit of the glycine receptor confirmed the diagnosis. A repeated needle muscle biopsy demonstrated mild myopathic changes, which we considered to be secondary to increased muscle tone. This case highlights the diagnostic difficulties of hyperekplexia, particularly in sporadic cases with unusual presentation.- - - - - - - - - - ranking = 3keywords = muscle (Clic here for more details about this article) |
5/49. Rapidly progressive polymyositis with elevated antiacetylcholine receptor antibody activity.We report a 51-year-old woman with polymyositis accompanied by a high titer of antiacetylcholine receptor antibody. The patient presented with weakness of grip strength followed by rapidly progressive dyspnea, which required mechanical ventilation. She was treated with a glucocorticoid and came off the respirator one week later. Antiacetylcholine receptor antibody activity was elevated in the acute phase and decreased during recovery, although other signs of myasthenia gravis were negative. This patient suggested that in cases of rapidly progressive bulbar palsy and limb muscle weakness, it is necessary to include polymyositis associated with elevated antiacetylcholine receptor antibody activity in the differential diagnosis.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/49. Clonus: an unusual delayed neurological complication in electrical burn injury.patients surviving high-voltage electrical injury may have early and delayed sequelae. The most apparent neurological complications are known to be cerebral injury, spinal cord lesions, peripheral-nerve injuries and motor neuropathies. In this study, clonus, which is an unusual late neurological sequela in an electrical burn patient and presented as series of rhythmic, monophasic contractions and relaxations of a group of muscles, is presented. Possible mechanisms of this unusual late sequela and the clinical outcome of the patient are discussed. ankle and patellar clonus was observed in 4 patients and uvular clonus in 1 patient. Clonus started 3 weeks following the injury in our patients and disappeared over a period of 1 yr in 2 patients, and did not disappear in the remaining 2 patients. In the current literature, this is the first report, which presents an unusual sequela following electrical injury. Clonus should also be considered a specific type of neurological sequela following high- or super-voltage electric injury. This may help to inform the patients in the postinjury period and to improve the efficacy of the rehabilitation of the victims.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
7/49. Use of the orbicularis oculi muscle flap for severe Marcus Gunn ptosis.The authors present a 22-year-old man with severe unilateral congenital blepharoptosis associated with Marcus Gunn (jaw-winking) syndrome. The best conventional treatment was levator excision to eliminate the synkinetic reflex and fascia lata brow suspension. However, the previous surgery had some disadvantages. Therefore, the authors report the use of the orbicularis oculi muscle flap to elevate dynamically the ptotic eyelid and to eliminate the synkinetic reflex without levator resection. The postoperative result was both functionally and cosmetically satisfactory after 1 year of follow-up.- - - - - - - - - - ranking = 5keywords = muscle (Clic here for more details about this article) |
8/49. Hyperreflexia in axonal guillain-barre syndrome subsequent to campylobacter jejuni enteritis.We describe a patient with the acute motor axonal neuropathy (AMAN) form of guillain-barre syndrome (GBS), who showed generalized hyperreflexia. A 24-year-old man developed acute paralysis following campylobacter jejuni enteritis. He showed exaggerated tendon reflexes with abnormal reflex spread to other segments, and was initially diagnosed as having post-infectious myelitis. Nerve conduction studies showed motor axonal degeneration (the AMAN pattern), and increased soleus h-reflex amplitudes. His serum was positive for IgG antibodies to gangliosides GM1b and GalNAc-GD1a. He was treated with plasmapheresis, resulting in rapid recovery. Hyperreflexia was still present 12 months after onset when muscle strength was completely normal. This case provides further evidence that patients with AMAN can develop increased motor neuron excitability, and possible mechanisms for the hyperreflexia are discussed.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
9/49. Radiological and manometric diagnosis of cricopharyngeal dysphagia in a Japanese encephalitis survivor.Japanese encephalitis (JE) is endemic throughout most of the western Pacific region where taiwan is located. About half the survivors are left with neurological damages. We report a 55-year-old male who survived from JE and was left with sequela of parkinsonism and severe swallowing disorder. Later, it was proved to be cricopharyngeal dysphagia (CPD) using esophagogram and manometry, which disclosed involuntary hypertonic and hyperreflexic cricopharyngeal muscle contraction. CPD, a life-threatening neurological sequel of JE, has never been reported in the JE survivors before and possibly results from disseminated lesions over pyramidal and extrapyramidal systems.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
10/49. Cervical cord tethering due to split cord malformation at the cervico-dorsal junction presenting with self-mutilation of the fingers.An unusual case of cervical spinal cord tethering with diplomyelia is described. A 12-month old female presented with self-mutilation of the fingers due to sensory loss in the hands, absent reflexes, poor muscle tone, and reduced distal upper-limb movements. There was a deep skin dimple overlying the T1 spinous process. Imaging showed angulation of the lower cervical cord and an operation revealed a low cervical meningocele and a split cord malformation with tethering of one half of the cord; the cord was untethered. In this report the literature is reviewed.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
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