Abnormalities of somatosensory and motor evoked potentials in adrenomyeloneuropathy: Comparison with magnetic resonance imaging and clinical findings

Restuccia, Domenico; Lazzaro, Vincenzo Di; Valeriani, Massimiliano; Oliviero, Antonio; Pera, Domenica Le; Barba, Carmen; Cappa, Marco; Bertini, E.; Tonali, P.

doi:10.1002/(sici)1097-4598(199710)20:10<1249::aid-mus7>3.0.co;2-c

Cited by 14 publications

(9 citation statements)

References 35 publications

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“…Since symptomatic female carriers of X-ALD resemble male AMN patients clinically, electrophysiological evidence of pyramidal involvement would also be expected in female heterozygotes. In accordance with this hypothesis, lower limb MEPs were abnormal in all patients whilst upper limb MEPs were consistently normal confirming the predominance of lower limb pyramidal tract involvement in female carriers of X-ALD [36]. Abnormal MEPs have not only been demonstrated in clinically symptomatic but also in clinically asymptomatic female carriers [36].…”

Section: Discussion S Clinical Findingssupporting

confidence: 87%

“…Correspondingly, MEPs were also abnormal in the only patient without clinical evidence of central motor pathway pathology. Apart from central motor tracts, somatosensory pathways are most frequently affected in symptomatic female carriers and male AMN patients [2,30,32,36,44,45]. Similar to the MEP findings, our data confirm a predominant somatosensory tract involvement to the lower extremities [22].…”

Section: Discussion S Clinical Findingssupporting

confidence: 77%

“…In accordance with this hypothesis, lower limb MEPs were abnormal in all patients whilst upper limb MEPs were consistently normal confirming the predominance of lower limb pyramidal tract involvement in female carriers of X-ALD [36]. Abnormal MEPs have not only been demonstrated in clinically symptomatic but also in clinically asymptomatic female carriers [36]. Correspondingly, MEPs were also abnormal in the only patient without clinical evidence of central motor pathway pathology.…”

Section: Discussion S Clinical Findingsmentioning

confidence: 99%

“…Whilst the majority of symptomatic female carriers exhibit only mild neurological dysfunction, rare cases of rapidly progressive CNS demyelination have also been reported [40,41]. Most reports on neurological involvement in female carriers of X-ALD have been based on clinical examination [30,31], and only few studies have investigated systematically the extent and distribution of nervous system involvement by electrophysiology and magnetic resonance imaging (MRI) [35,36]. Moreover, recent studies using magnetic resonance spectroscopy (MRS) have demonstrated abnormal metabolite ratios of N-acetylaspartate (NAA)/choline (Cho) in affected and non-affected cerebral white matter of X-ALD patients, indicating a high sensitivity of MRS in the detection of cerebral involvement in X-ALD [24,43].…”

Section: Introductionmentioning

confidence: 99%

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Phenotype assignment in symptomatic female carriers of X-linked adrenoleukodystrophy

Schmidt¹,

Träber

Block

et al. 2001

Journal of Neurology

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However, only very few studies have systematically investigated the extent and distribution of nervous and endocrine system involvement in female carriers of X-ALD. To define the phenotype in symptomatic female carriers of X-ALD we performed a prospective study including eight symptomatic women who were followed for a mean period of 3.4+/-1.8 years (range 1-6) using standardized clinical examination protocols, magnetic resonance imaging and spectroscopy, evoked potential studies including visual, brainstem auditory, somatosensory and magnetic evoked potentials, neurographic recordings and endocrine studies. Spastic paraparesis and decreased vibration sense in the lower extremities were the most frequent clinical findings. Slightly hyperintense symmetric parieto-occipital white matter lesions on magnetic resonance imaging were detectable in two of seven cases, and the N-acetylaspartate/choline ratios on magnetic resonance spectroscopy were decreased in three of seven patients. P40 latencies were abnormal in all patients, and central motor conduction times to the lower extremities in seven of eight patients. Prolonged latencies of brainstem auditory evoked potential waves III-V or interpeak latencies of waves I-III, I-V and III-V were detectable in all patients. The degree of walking impairment was positively correlated with the duration of clinical disease (r=0.58, P < 0.05) and inversely correlated with the N-acetylaspartate/choline ratios (r=0.85; P < 0.05). Neurographic recordings revealed only subtle abnormalities, suggesting that nervous system involvement in symptomatic female carriers of X-ALD is confined mainly to the central nervous system. No evidence of adrenal insufficiency was detected in any of the patients.

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Section: Discussion S Clinical Findingssupporting

confidence: 87%

Section: Discussion S Clinical Findingssupporting

confidence: 77%

Section: Discussion S Clinical Findingsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Phenotype assignment in symptomatic female carriers of X-linked adrenoleukodystrophy

Schmidt¹,

Träber

Block

et al. 2001

Journal of Neurology

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“…Adrenomyeloleukodystro− phie lassen sich gleichfalls bei fraktionierter Ableitung nach pe− ripherer Nervenstimulation neben peripheren Latenzverzöge− rungen deutliche Verlängerungen der zentralen Überleitungszeit der SEP dokumentieren. Ebenso sind hochgradige Verzögerun− gen der CMCT sowie der fraktioniert zu erfassenden peripheren motorischen Leitungszeit beschrieben[56,57]. Auch bei Strah− lenmyelopathien können in variabler Ausprägung MEP− und SEP− Veränderungen (Leitungsverzögerung und/oder Amplitudenre− duktion) beobachtet werden[29].Gleichfalls individuell sehr unterschiedliche SEP−Befunde finden sich in Abhängigkeit vom Krankheitsstadium bei Patienten mit Syringomyelie.…”

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Evozierte Potenziale in der Diagnostik spinaler Erkrankungen

Schwenkreis¹,

Tegenthoff²

2005

Klin Neurophysiol

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Zusammenfassung Für die Funktionsbeurteilung spinaler Bahnsysteme eignen sich grundsätzlich sowohl motorisch (MEP) als auch somatosenso− risch (SEP) evozierte Potenziale. Aufgrund der Physiologie der Reizleitung liefern die MEP dabei Informationen über ventrale Rückenmarksanteile (Pyramidenbahn, Vorderhorn, Vorderwur− zelaustrittszone), SEP über dorsale Anteile des Rückenmarks− querschnittes (Hinterwurzeleintrittszone, Hinterstränge). Mög− liche klinische Fragestellungen beim Einsatz der evozierten Po− tenziale umfassen dabei die Objektivierung von Läsionen bei akuten spinalen Erkrankungen, aber auch eine vertikale bzw. ho− rizontale Lokalisationsdiagnostik sowie mögliche prognostische Aussagen. Weitere Indikationen umfassen die Entdeckung sub− klinischer Läsionen sowie die Verlaufsbeurteilung bei eher chro− nischen spinalen Erkrankungen. Eine vertikale Lokalisationsdi− agnostik ist dabei durch die Wahl geeigneter Ableit− und Stimu− lationsorte sowohl für die MEP als auch für die SEP in gewissen Grenzen möglich. Dargestellt werden Anwendung und typische Befundkonstellationen bei akuten und chronischen Rücken− markstraumen, spinalen Raumforderungen einschließlich zervi− kaler Myelopathie, vaskulären und entzündlichen Myelopathien, psychogener Querschnittssymptomatik sowie einer Reihe von selteneren spinalen Erkrankungen. Dabei wird gezeigt, wie SEP und MEP bei Berücksichtigung der klinischen Symptomatik so− wie der methodischen Einschränkung einer fehlenden ätiologi− schen Spezifität der Befunde einen wichtigen Beitrag zur Diag− nostik und Differenzialdiagnostik spinaler Erkrankungen liefern können. AbstractMotor (MEP) and somatosensory (SEP) evoked potentials can be used to assess the functionality and integrity of different ascend− ing and descending volleys of the spinal cord. Whereas MEP pro− vide information about ventral structures of the spinal cord (pyramidal tract, ventral horn), the registration of normal SEP requires an intact function of the dorsal column and dorsal root entrance zone. Evoked potentials can be used in clinical practice to objectify acute spinal lesions, to provide a horizontal and ver− tical localisation of the lesion or to obtain information about the prognosis of a spinal lesion. Further applications are the detec− tion of subclinical lesions and the development of the lesions in chronic diseases involving the spinal cord. A vertical localisation of spinal lesions can be performed by selecting multiple individ− ually adapted recording and stimulation sites. We present the re− sults in acute and chronic stages of spinal cord injury, in spinal tumours or cervical myelopathy, in ischaemic or inflammatory myelopathy, in psychogenic paraplegia and in a number of other infrequent disorders affecting the spinal cord. It is shown how SEP and MEP can provide useful information complementary to the clinical findings in the diagnosis and differential diagnosis of spinal cord lesions of different origin, with respect to the lack of etiological specificity, which should be considered when inter− ...

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