1/22. Symptoms of recurrent intrathecal baclofen withdrawal resulting from drug delivery failure: a case report.A 24-yr-old, completely (T8) paraplegic male patient presenting with severe spasticity had a drug administration device implanted in April 1991 for continuous intrathecal administration of baclofen. After a period of remarkable improvement in both the spasticity level and his quality of life, the patient experienced several short-lasting episodes of increased spasticity, with severe spasms. Among the possible causes of these deleterious episodes were microcrystalluria, obstipation, a decubitus ulcer, a foreign body in the buttocks, drug tolerance to baclofen, electromagnetic interference, and erroneous filling and programing of the pump. The catheter was the most common source of intrathecal baclofen withdrawal symptoms and had to be changed four times in 5 yr. Intrathecal baclofen administered through an implantable drug administration device is a highly effective but complex and expensive procedure that requires careful patient selection and close monitoring by highly qualified and well-trained health professional. Withdrawal symptoms may be related to noncompliance on the part of the patient, erroneous filling or programing of the pump, depletion of the battery, random component failure, concomitant illness, drug tolerance, or advancement of the disease itself. When failure of the device is suspected, substitution with oral baclofen is recommended until a full work-up is performed to determine the defect.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/22. Motor control testing of upper limb function after botulinum toxin injection: a case study.OBJECTIVE: To evaluate changes in upper extremity function in a hemiparetic patient after treatment with botulinum toxin (BTX) using motor-control testing (MCT) techniques. DESIGN: Interventional with longitudinal study, open label. SETTING: A children's hospital and a motor-control laboratory at a major academic center. PARTICIPANTS: A 16-year-old male with right hemiparetic cerebral palsy and a healthy 12-year-old control subject. INTERVENTIONS: BTX injections to the elbow and wrist flexors. MAIN OUTCOME MEASURES: MCT was used to examine 4 upper extremity movements: forward reach, bilateral rhythmic movements (both muscle homologous and direction homologous), isometric pinch, and hand tapping. The patient was tested before treatment and at 2, 4, 6, 12, 18, and 24 weeks after treatment. In addition, range of motion (ROM), the Ashworth scale of spasticity, Functional Independence Measure, and the mobility and activities of daily living (ADL) sections of the Pediatric Evaluation of the Disability Inventory were performed. RESULTS: Forward reach demonstrated little change initially despite patient reports of "feeling looser." Improvement was noted after 18 weeks, but returned to baseline level at 24 weeks. Bilateral rhythmic movements also showed slight improvement at 18 weeks. Pinch force increased significantly after 2 weeks, but declined again at 6 weeks. Improvements occurred in ROM and the Ashworth rating of spasticity, but were not temporally associated with each other or with MCT results. Functional assessment data did not change during the study period. CONCLUSIONS: Improvements in more complex motor tasks were noted after significant delay from the time of treatment, while simpler tasks demonstrated a more rapid improvement, followed by a rapid return to baseline levels. This case suggests that MCT techniques can provide quantitative and qualitative data, which can add new information about upper extremity motor disability and the outcome of treatment.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/22. Powered mobility and preschoolers with complex developmental delays.OBJECTIVE: The purpose of this study was to explore the effects of a powered mobility riding toy on the participation behaviors of young children with complex developmental delays. METHOD: A single-subject withdrawal design was used to study the effects of powered mobility on child-initiated movement occurrences, initiation of contact with others, and affect. The participants were two young children with complex developmental delays, including spastic quadriplegia. The intervention consisted of having the children use a powered mobility riding toy in their school settings during gym class and outdoor recess. RESULTS: Primary findings were that use of the powered mobility riding toy (a) increased the number of self-initiated movement occurrences; (b) appeared to have some effect on initiation of contacts with adults and, for one child, negative adult initiations and positive peer initiations; and (c) did not have a clear impact on the amount of positive affect. CONCLUSION: For some young children with severe motor impairments and developmental delay, use of a powered mobility device may increase self-initiated movement occurrences during free play.- - - - - - - - - - ranking = 6keywords = complex (Clic here for more details about this article) |
4/22. multiple sclerosis and oral care.multiple sclerosis is a complex neurological condition affecting sensory and motor nerve transmission. Its progression and symptoms are unpredictable and vary from person to person as well as over time. Common early symptoms include visual disturbances, facial pain or trigeminal neuralgia and paraesthesia or numbness of feet, legs, hands and arms. These, plus symptoms of spasticity, spasms, tremor, fatigue, depression and progressive disability, impact on the individual's ability to maintain oral health, cope with dental treatment and access dental services. Also, many of the medications used in the symptomatic management of the condition have the potential to cause dry mouth and associated oral disease. There is no cure for multiple sclerosis, and treatment focuses on prevention of disability and maintenance of quality of life. Increasingly a multi-disciplinary team approach is used where the individual, if appropriate his/her carer, and the specialist nurse are key figures. The dental team plays an essential role in ensuring that oral health impacts positively on general health.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/22. Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type.BACKGROUND: Recently, six molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only isolated cases of the rare VV1 type have been reported so far. OBJECTIVE: To describe the clinical characteristics and neuropathologic lesion profiles in nine cases. methods: In the years 1993 until late 2003, 571 definite neuropathologically confirmed cases of sporadic CJD were identified in germany. Of these, nine were homozygous for valine and displayed type 1 of the pathologic PrPSc in the brain (VV1 type). RESULTS: The authors describe eight men and one woman belonging to the VV1 type. All patients were relatively young at disease onset (median 44 years vs 65 years in all sCJD) with prolonged disease duration (median 21 months vs 6 months in all sCJD). During the initial stages, their main clinical signs were personality changes and slowly progressive dementia as well as focal neurologic deficits. None of the nine VV1 patients had periodic sharp-wave complexes (PSWCs) in the EEG. Only two out of seven displayed the typical signal increase of the basal ganglia on MRI, whereas signal increase of the cortex was seen in all patients. The 14-3-3 protein levels were elevated in CSF in all cases tested. CONCLUSIONS: The clinical diagnosis of the VV1 type of sCJD can be best supported by the 14-3-3 test and cortical signal increase on MRI. Because of the young age at onset vCJD is sometimes suspected as a differential diagnosis. MRI plays an important role in differentiating these two disease types and should be performed early during the disease course.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/22. Epileptic seizures in the 4p- syndrome: report of two cases.We report ictal phenomena in two patients with the 4p- syndrome captured on simultaneous video-EEG monitor. One patient, diagnosed as having partial epilepsy, had complex partial seizures and hemiconvulsive status epilepticus. This was associated with more severe mental retardation. The second patient was diagnosed as having the West syndrome and exhibited tonic spasms with a cluster formation. We conclude that various types of epileptic seizures may occur in patients with the 4p- syndrome, including grand mal and myoclonic seizures.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/22. Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. I. Clinical and endocrinologic aspects.We have studied four unrelated males with a heritable disorder that we term adrenomyeloneuropathy; limited clinical information is available on a fifth case. All had adrenal insufficiency beginning in childhood and developed progressive spastic paraparesis in the third decade. hypogonadism of variable severity was present in all four cases appropriately examined. Neurologic features included peripheral neuropathy, impotence, and sphincter disturbances. Late manifestations were cerebellar dysfunction in one case and dementia and hemiparesis in another. A family history of adrenal disease or spastic paraparesis was present in two cases and absent in one; in the other two, no family history was available. Although males are predominantly affected, the mode of inheritance is uncertain. Adrenomyeloneuropathy probably represents a clinically and genetically distinct variant of childhood adrenoleukodystrophy.- - - - - - - - - - ranking = 956503.25925266keywords = adrenomyeloneuropathy, adrenoleukodystrophy (Clic here for more details about this article) |
8/22. Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. II. General pathologic, neuropathologic, and biochemical aspects.Histopathologic features were studied in postmortem examination of two men with adrenomyeloneuropathy, and biochemical studies were performed on one of these individuals. The histopathologic picture of one case included dying-back features in the nervous system and lamellar cytoplasmic inclusions in the brain, adrenal gland, and testis similar to those in adrenoleukodystrophy. Biochemical studies of the cerebral white matter of this individual revealed increased amounts of long-chain saturated fatty acids in cholesterol esters, an abnormality characteristic of adrenoleukodystrophy. Despite differences in clinical presentation and neuropathology, adrenomyeloneuropathy probably represents a distinct variant of adrenoleukodystrophy.- - - - - - - - - - ranking = 1681555.6264706keywords = adrenomyeloneuropathy, adrenoleukodystrophy (Clic here for more details about this article) |
9/22. Multimodality evoked potentials in patients and carriers with adrenoleukodystrophy and adrenomyeloneuropathy.A combination of brain-stem auditory evoked potentials (BAEPs), short latency somatosensory evoked potentials (SEPs) and pattern reversal visual evoked potentials (VEPs), were studied in two patients with adrenoleukodystrophy (ALD) and one patient with adrenomyeloneuropathy (AMN), as well as in one female carrier of each of the respective diseases. Abnormalities in at least 1 of the 3 evoked potentials were found in every case, including the carriers of ALD and AMN. The two most common findings were prolongation of the I-V interval of the BAEP and the N13-N20 interval of the SEP. These abnormalities were recorded either alone or in combination in all 5 cases. This finding suggests delayed conduction time in the central sensory pathways in both diseases, probably due to demyelination. The remarkable result, which distinguished AMN from ALD, even in their respective carriers, was delay of the N9 latency of the SEP, indicating slowing in conduction velocity of the peripheral nerve. Multimodality evoked potentials are useful not only in raising the detection rate for abnormal findings, but also in providing additional information about the functional state of separate afferent pathways. It is also of value in detecting and differentiating the carriers of ALD and AMN.- - - - - - - - - - ranking = 3239510.3493653keywords = adrenomyeloneuropathy, adrenoleukodystrophy (Clic here for more details about this article) |
10/22. Implantation of a recurrent laryngeal nerve stimulator for the treatment of spastic dysphonia.Spastic dysphonia, a rare speech disorder, is characterized by strained phonation with excessively adducted vocal cords. recurrent laryngeal nerve section, botulinum toxin injection into the vocalis-thyroarytenoid muscle complex, and other techniques have been used to treat this disorder. We have used percutaneous electrical stimulation of the recurrent laryngeal nerve with good results. Previous dog studies demonstrated the relative safety of an implantable recurrent laryngeal nerve stimulator. In this study, we directly stimulated the recurrent laryngeal nerve and vagus nerve in a dog without change in cardiorespiratory status. A Medtronic peripheral nerve stimulator was implanted in a patient with abductor spastic dysphonia. The cuff electrode was positioned around the recurrent laryngeal nerve and stimulation resulted in improvement in her voice. Extensive cardiopulmonary monitoring did not reveal any adverse response to stimulation and there was no discomfort to the patient. On the basis of the good results of this preliminary study, further study with long-term follow-up is under way.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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