M de Carvalho

..We conclude that neck weakness is a clinical sign that indicates a poor prognosis, and the SCM CMAP can contribute to respiratory function evaluation in ALS patients...

..Respiratory function tests are used to detect early signs of hypoventilation in amyotrophic lateral sclerosis (ALS). However, there are few studies of the predictive value of these tests in detecting hypoventilation...

..We aimed to investigate firing rate in different disorders, testing the same target muscle with normal strength, to evaluate possible application in diagnosing upper motor neuron (UMN) lesion...

..The NSprogressed in the first year following LT, and subsequently it did not increase significantly. LT changed the natural course of FAP-I...

..To evaluate the pattern of degeneration of lower motor neuron progression in ALS in relation to the contralateral and ipsilateral corticospinal innervation of the tested muscles...

..We speculate that they have a central respiratory dysfunction and deserve special care...

..Our patient developed a limited form of anterior horn cell degeneration perhaps representing a syndrome transitional with amyotrophic lateral sclerosis. Cramp-FS merits more detailed study...

..We present new data demonstrating that this effect is also observed in fatigued muscle. We conclude that CSP can be considered a surrogate marker of the effect of caffeine in the brain, in particular of its central ergogenic effect...

..This observation raises the possibility that lower motor neuron hyperexcitability may precede motor neuron death in motor neuron disease...

..This can be important for prognosis, in particular when liver transplantation is considered for treatment...

..The patient illustrates the confusion and difficulty in categorising an usual clinical syndrome...

..The CSP may be a useful variable in following UMN dysfunction in clinical practice and in clinical trials...

..Axial muscles denervation is related to diaphragm denervation and therefore to poor respiratory function in ALS. We suggest that medially located lower motor neurons are affected concurrently in ALS...

..On assessing progression at 3, 6, and 12 months, MUNE, NI, and MVIC-ADM showed the highest rate of change. The NI is a potentially useful new neurophysiologic measurement...

..We conclude that clinical neurophysiological techniques should now be used in measuring change in clinical trials in ALS...

..These unique observations show that ALS commences at a defined time, and that there is early generalisation with an initial phase of rapid progression...

..We investigated how frequently an initial electrophysiological (EMG) evaluation, performed in ALS patients, revealed no fibs-sw...

..It is suggested that early in ALS the corticomotor threshold (CMT) is low, but increases with increasing duration of the disease...

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..A preserved SPSP should not exclude a CPN neuropathy...

..The changes in map parameters were attributed primarily to loss of cortical cells. These results indicate that map parameters may be more sensitive to cortical neuronal loss than other TMS parameters...

..The complex relation of ALS with RSD is discussed. In all ALS patients pain followed by further loss of function should prompt a search for RSD...

..The aim is to review recent publications on amyotrophic lateral sclerosis (ALS)...

..These results confirm the value of paraspinal MUP analysis in the diagnosis of ALS...

..The neurologist must be aware of this association to guide the affected patient to a correct treatment...

..The typically transient and quickly reversible nerve dysfunction caused by ion channel blockade is reported...

..In conclusion, the results of this study show that memantine is well tolerated and safe in ALS patients. We did not observe any evidence of efficacy for memantine but we cannot exclude a positive outcome on survival...

..A wide discussion among neurophysiologists from different schools could create a sound consensus on early diagnosis of MND-ALS...

..Somatosensory evoked potentials, transcranial magnetic stimulation, and MRI of the cervical and thoracic spine were normal. Tremor persisted in REM and non-REM sleep. These findings suggest a peripheral generator...

..We aim to describe the clinical and neurophysiological characteristics of a group of patients with a clinical diagnosis of deep palmar branch lesion of the ulnar nerve. We report the clinical and neurophysiological outcome...

..The aim of the present study was to determine if bulbar-onset patients have an earlier respiratory impairment as compared with the spinal-onset ones...

..02) and a significant difference in the slope of the RFT (P<0.008) were observed in the treated group, suggesting that exercise may be beneficial in ALS patients once Bipap is used to control peripheral and muscle oxygenation...

..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...

..To test the influence of caffeine on the lower and upper motor neuron excitability...

..One year later, the patient developed dysautonomic symptoms. Following confirmation of adrenal insufficiency, a diagnosis of Allgrove syndrome was made. This is a rare case, and we emphasize its clinical similarity with ALS...

..The reliability of these neurophysiological and strength measurements in the same nerve/muscle system suggests they may be useful in comparing the dynamics of weakness and neurophysiological change in neuromuscular disease...

..Increased CK can be useful in the differential diagnosis of patients with lower motor neuron disorders...

..We suggest that expressing the data as an index and utilising the MEP/M wave amplitude ratio as a variable is a sensitive method for detecting UMN abnormality in ALS in particular in early affected muscles...

..The ADM CMAP is asymmetric in upper-limb-onset patients. Unilateral stimulation is sufficient to monitor the phrenic nerve response; its degree of CMAP decrease does not correlate with other cervical muscle involvement...

..The abnormalities were more frequent in very weak limbs, and could be ascribed to lack of use. CONCLUSION: Although significant sudomotor hypofunction does not occur in ALS, mild subclinical changes can be observed in weak limbs...

..The aim of this study is to describe a method for recording compound muscle action potential (CMAP) from the sternocleidomastoid (SCM) in response to accessory-nerve stimulation...

..A larger number of responding motor units in these weak limbs with UMN signs was observed. CONCLUSION: F-wave excitability is largely dependent on the size of the lower motor neuron pool...

..Phrenic nerve motor amplitude (Diaphr Ampl) is predictive of hypoventilation in amyotrophic lateral sclerosis (ALS). We aimed to evaluate its change over disease course and to correlate it to other measurements...

..Reliable quantitative methods that could allow the determination of early changes in the peripheral nerve function are essential. Our aim was to find sensitive neurophysiological markers in FAP-I...

..Furthermore, numerical results for the diaphragmatic floor under pressure and active contraction in normal and pathological cases are presented...

..MOP and ET should also be used in evaluation protocols of ALS/MND...

..The second case illustrates that the temporal dispersion of the motor responses can be misleading, and EMG of diaphragm should be performed to confirm the loss of motor units...

..This case confirms that TTR mutation screening should be considered in patients with a clinical disorder consistent with amyloid neuropathy even in the absence of a family history...

..This report documents the lesion of the motor nerve fibers in this disease, as well as the preservation of the autonomic nervous system function, therefore suggesting that HSNA is an inappropriate name for this disorder...

..In conclusion, presymptomatic gene testing for FAP may have a positive impact on candidate quality and prolongation of life, and on the future of disease studies...

..002). We concluded that NPO is a valuable screening test to establish the need for NIV. Our results also suggest that the early diagnosis of RI and the early use of NIV increase patient compliance with NIV...

..The marked variability in progression of amyotrophic lateral sclerosis (ALS) requires large numbers of patients to detect a significant effect in current clinical trial designs...

..Neurophysiological measurements were similar. FAP is a cause of neuropathy in elderly patients, in particular in those presenting with neuropathic pain...

..ALS, particularly when of bulbar onset, can cause respiratory apraxia and EMG of the respiratory muscles can be useful to detect this condition...

..Multifocal motor neuropathy should be considered when there is progression, even years after onset...

..The Finnish mutation of gelsolin protein (G654A) was detected in five family members. The utility of neurophysiological testing in the evaluation and follow-up of this type of amyloidosis is discussed...

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..In the past some trials may have failed to demonstrate a treatment effect because the chosen endpoint measures and the trial design were inappropriate...

..Overall the pattern of change resembled that of ALS, although some patients progressed very slowly. Axial onset, however, predicts the early onset of respiratory failure, and a poor prognosis...

..We conclude that the NI, which requires no special technology and no new clinical or technical skills to use, is sensitive to change, and therefore may be useful in clinical trials, as well as in a clinical setting...

..1996)--especially when associated with cryoglobulinemia. We report, for the first time, the association between pure motor-axonal polyneuropathy and HCV infection without cryoglobulinemia...

..Based in these cases, a thyrotoxic myopathy brief comment is made, as in spite of being frequent, these cases presented diagnosis difficulties...

..Our report shows that FAP can develop in a recipient of an FAP liver. This suggests that careful longitudinal study is required to evaluate the risk of FAP polyneuropathy in patients who undergo domino liver transplantation...

..PNO and polysomnography suggest that respiratory central drive dysfunction can occur when upper motor neurons are severely affected, in PLS. However, we did not verify progression on follow-up...

..To correlate repetitive nerve stimulation (RNS) decrement in different muscles with the predominant clinical presentation in myasthenia gravis (MG), and to study single fibre EMG (SFEMG) sensitivity in ocular MG...

..This case supports the role of muscle biopsy in patients that develop muscular necrosis while on statin treatment...

..However, it requires a process of adaptation not always achieved due to poor compliance. The role of telemonitoring of NIV is not yet established...

..Respiratory function was mildly affected. Cerebellar ataxia developed over time and the diagnosis of MJD was confirmed by genetic studies. MJD should be considered in the differential diagnosis of MND...

..Methods for detection of upper motor neuron abnormality appear sensitive but require further study, particularly regarding their value when clinical signs of upper motor neuron lesion are uncertain...

..Sialorrhea is a disabling problem in bulbaronset amyotrophic lateral sclerosis (ALS). Botulinum toxin (BTX) type A and B have been proposed as alternatives to traditional treatments...

..Patient 1 shows that PML is a possible cause for pseudobulbar syndrome, and our second patient demonstrates that ALS may also occur by chance in patients with HIV infection...

..We discuss the association between ALS and ocular myasthenia gravis in these cases...

..Young-onset ALS seems to be a distinct clinical syndrome but its aetiological background is largely unknown...