Visually evoked potentials in respiratory chain disorders

Finsterer, Josef

doi:10.1034/j.1600-0404.2001.00167.x

Cited by 14 publications

(6 citation statements)

References 12 publications

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“…Normal biochemical findings, and the negative screening for mtDNA mutations do not exclude mitochondriopathy because reduction of respiratory chain activity is dependent on the rate of heteroplasmy, and because the muscle was screened for only 7 mtDNA mutations. Bilaterally prolonged VEPs are a frequent finding in mitochondriopathy and cannot be explained by a unilateral cataract (Finsterer, 2001). The late onset does not exclude mitochondriopathy because non-neurological manifestations were already present at an age at which mitochondriopathy usually starts (DiLazzaro et al, 1997;Van Goethem et al, 1957).…”

Section: Discussionmentioning

confidence: 97%

Unilateral Compression Neuropathy of the Hypoglossal Nerve Due to Head Suspension Orthosis in Mitochondriopathy

Finsterer

Hess

2004

International Journal of Neuroscience

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An 85-year-old woman with multisystem mitochondriopathy experienced tension headache, cervical pain, torque head-posture, and vertigo since 1980 for which she was continuously wearing a head-suspension-orthosis- since 1990. Since 1996 she developed severe left-sided weakness and wasting of the tongue. Needle-EMG of the left genioglossus muscle revealed abnormal spontaneous activity and reduced interference-pattern. No morphological alterations in the anatomical course of the hypoglossal nerve were found. Severe, unilateral weakness and wasting of the tongue was interpreted due to chronic compression of the hypoglossal nerve by long-standing use of a head-suspension-orthosis for cervical pain from cervical muscle weakness and resulting spinal degeneration.

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Section: Discussionmentioning

confidence: 97%

Unilateral Compression Neuropathy of the Hypoglossal Nerve Due to Head Suspension Orthosis in Mitochondriopathy

Finsterer

Hess

2004

International Journal of Neuroscience

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“…In most adult cases, MCPs are multisystem disorders, affecting the peripheral nervous system, central nervous system, endocrinium, heart, gastrointestinal tract, eyes, ears, kidneys, dermis, bone marrow or cartilage (20, 22). Central nervous system manifestations of MCPs are stroke, epilepsy, dementia, ataxia, myocloni, prolonged latencies of visually evoked potentials (20, 23), unspecific white matter lesions or basal ganglia calcification (2, 20, 24). In the present investigation the diagnosis of MCP was based upon clinical and muscle biopsy findings.…”

Section: Discussionmentioning

confidence: 99%

Parkinson syndrome as a manifestation of mitochondriopathy

Finsterer

2002

Acta Neurologica Scandinavica

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“…Visually evoked potentials (VEPs) may show prolonged P100 latencies, even in the absence of morphological abnormalities (150), suggesting that VEPs should be part of the diagnostic work‐up in patients with suspected MCD (151). Acoustically evoked potentials (AEPs) may demonstrate cochlear nerve involvement in LHON (152).…”

Section: Diagnosis Of Cns Involvement In Mcdsmentioning

confidence: 99%

Central nervous system manifestations of mitochondrial disorders

2006

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The central nervous system (CNS) is, after the peripheral nervous system, the second most frequently affected organ in mitochondrial disorders (MCDs). CNS involvement in MCDs is clinically heterogeneous, manifesting as epilepsy, stroke-like episodes, migraine, ataxia, spasticity, extrapyramidal abnormalities, bulbar dysfunction, psychiatric abnormalities, neuropsychological deficits, or hypophysial abnormalities. CNS involvement is found in syndromic and non-syndromic MCDs. Syndromic MCDs with CNS involvement include mitochondrial encephalomyopathy, lactacidosis, stroke-like episodes syndrome, myoclonic epilepsy and ragged red fibers syndrome, mitochondrial neuro-gastrointestinal encephalomyopathy syndrome, neurogenic muscle weakness, ataxia, and retinitis pigmentosa syndrome, mitochondrial depletion syndrome, Kearns-Sayre syndrome, and Leigh syndrome, Leber's hereditary optic neuropathy, Friedreich's ataxia, and multiple systemic lipomatosis. As CNS involvement is often subclinical, the CNS including the spinal cord should be investigated even in the absence of overt clinical CNS manifestations. CNS investigations comprise the history, clinical neurological examination, neuropsychological tests, electroencephalogram, cerebral computed tomography scan, and magnetic resonance imaging. A spinal tap is indicated if there is episodic or permanent impaired consciousness or in case of cognitive decline. More sophisticated methods are required if the CNS is solely affected. Treatment of CNS manifestations in MCDs is symptomatic and focused on epilepsy, headache, lactacidosis, impaired consciousness, confusion, spasticity, extrapyramidal abnormalities, or depression. Valproate, carbamazepine, corticosteroids, acetyl salicylic acid, local and volatile anesthetics should be applied with caution. Avoiding certain drugs is often more beneficial than application of established, apparently indicated drugs.

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Visually evoked potentials in respiratory chain disorders

Abstract: VEPs prove useful to detect clinical or subclinical impairment of the optical tract in patients with RCDs. In the majority of the cases, the P100 latencies are prolonged while the P100/N145 amplitude remains normal.

Cited by 14 publications

References 12 publications

Unilateral Compression Neuropathy of the Hypoglossal Nerve Due to Head Suspension Orthosis in Mitochondriopathy

Unilateral Compression Neuropathy of the Hypoglossal Nerve Due to Head Suspension Orthosis in Mitochondriopathy

Parkinson syndrome as a manifestation of mitochondriopathy

Central nervous system manifestations of mitochondrial disorders

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