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Article ; Conference proceedings: Nutritional status of motor neuron diseases in relation to their disease duration

Sperfeld, A.D

2008

Abstract: During the course of amyotrophic lateral sclerosis/motor neurone diseases (ALS/MND) malnutrition occurs with a prevalence of 10–55 percent. Irrespective of the various causes of malnutrition (e.g. dysphagia, depression, disability to use the cutlery), an ...

Event/congress	Jahrestagung der Deutschen Gesellschaft für Neurologie (DGN), Hamburg, 2008
Abstract	During the course of amyotrophic lateral sclerosis/motor neurone diseases (ALS/MND) malnutrition occurs with a prevalence of 10–55 percent. Irrespective of the various causes of malnutrition (e.g. dysphagia, depression, disability to use the cutlery), an additional intrinsic hypermetabolism of unknown causes leading to an increase in resting energy expenditure. Because malnutrition and weight loss are independent significant prognostic factors for survival in ALS/MND, an adequate disease state adapted nutrition is an integral component of treatment in such diseases. Less is known about the nutritional status and the energy intake during the disease duration. This presentation gives an overview about a comparable simple nutritional assessment in AL/MND patients. Beside the estimation of disease related parameters (ALS-FRS-R, FEV1, presence of dysphagia) nutritional assessment includes the calculation of the Body Mass Index (BMI), estimation of the body composition by bioelectrical impedance analysis (BIA), and a standardised interview of the daily food composition (including artificial supplements and nutrition) will be done. One interesting point of this evaluation is to find out at which time point a change in or an additional nutrition becomes necessary. In conclusion, the improvement of nutritional status accounts for a shift from an attitude of nihilism to treatments that prolong survival and offer hope in ALS/MND patients.
Language	English
Publishing date	2008-09-02
Publishing place	Stuttgart ; New York
Document type	Article ; Conference proceedings
ZDB-ID	124980-0
ISSN	1438-9428 ; 0302-4350 ; 1431-4886
ISSN (online)	1438-9428
ISSN	0302-4350 ; 1431-4886
DOI	10.1055/s-0028-1086494
Database	Thieme publisher's database

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Article ; Online: Eine abszessverdächtige Raumforderung in der extraokulären Augenmuskulatur.

Schenke, T / Lehmann, G / Kunze, C / Bauer, M / Pazaitis, N / Odparlik, A / Krug, S / Sperfeld, A-D / Plontke, S K / Glien, A / Kisser, U

HNO

2020 Volume 68, Issue 9, Page(s) 698–702

Title translation	Suspected abscess formation in the inferior rectus muscle.
MeSH term(s)	Abscess ; Humans ; Oculomotor Muscles
Language	German
Publishing date	2020-04-02
Publishing country	Germany
Document type	Journal Article
ZDB-ID	200040-4
ISSN	1433-0458 ; 0017-6192
ISSN (online)	1433-0458
ISSN	0017-6192
DOI	10.1007/s00106-020-00856-7
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Article: Preclinical trials--an update on translational research in ALS.

Ludolph, Albert C / Sperfeld, A-D

Neuro-degenerative diseases

2005 Volume 2, Issue 3-4, Page(s) 215–219

Abstract: Translational research has become a strategy which describes the steps from in vitro experimental therapy, its translation into experimental animals and finally to humans. However, this translation of hypotheses from one of these levels to another faces ... ...

Abstract	Translational research has become a strategy which describes the steps from in vitro experimental therapy, its translation into experimental animals and finally to humans. However, this translation of hypotheses from one of these levels to another faces a number of difficulties which are currently of major interest for the development of therapeutics. In particular, in the previously untreatable motor neuron diseases, the steps from genes to transgenic animals and finally to the patient have proven difficult. High expectations have not met with the transfer of numerous therapies from experimental animals to men; there are many in- and outside the field which already question the value of animal models. It is the opinion of the authors that we should not dismiss the models before we have not defined generally accepted standards of protocols for therapeutic studies in experimental animals. Only if a generally agreed standardized and validated methodology in mice cannot predict the human response to therapeutics, an animal model should be abandoned. In contrast, the value of translating genetic findings to experimental animals and men is currently shown for motor proteins; in particular the functional impact of dynactin and dynein for the integrity of motor neurons of rodents and men are in the center of interest.
MeSH term(s)	Amyotrophic Lateral Sclerosis/drug therapy ; Animals ; Animals, Genetically Modified ; Disease Models, Animal ; Humans ; Mice ; Research Design
Language	English
Publishing date	2005
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2143569-8
ISSN	1660-2862 ; 1660-2854
ISSN (online)	1660-2862
ISSN	1660-2854
DOI	10.1159/000089628
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Article: Differential diagnosis in progressive infantile spastic tetraparesis.

Kühnlein, Peter / Sperfeld, A-D / Gdynia, H-J

European journal of medical research

2008 Volume 13, Issue 10, Page(s) 447–450

Abstract: Progressive infantile spastic tetraparesis spans a wide spectrum of partially rare differential diagnoses. Based on a clinical example the differential diagnostic thoughts are discussed in detail. Though juvenile motor neuron disease is a rare entity, it ...

Abstract	Progressive infantile spastic tetraparesis spans a wide spectrum of partially rare differential diagnoses. Based on a clinical example the differential diagnostic thoughts are discussed in detail. Though juvenile motor neuron disease is a rare entity, it has to be kept in mind for differential diagnostics in cases of slowly progressive spastic tetraparesis, especially when a pseudobulbar palsy or distal amyotrophies add to the clinical picture. Electromyography can be helpful for early detection of lower motor neuron involvement. The glutamate antagonist riluzole slows the disease progression, but a causal treatment is not available, yet. Therefore symptomatic treatment of disturbing symptoms like muscle cramps, spasticity, pseudobulbar affect, dyspnea or dysphagia are of major interest.
MeSH term(s)	Adult ; Age of Onset ; Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/genetics ; Amyotrophic Lateral Sclerosis/physiopathology ; Diagnosis, Differential ; Electromyography ; Guanine Nucleotide Exchange Factors/genetics ; Humans ; Male ; Quadriplegia/diagnosis ; Quadriplegia/genetics ; Quadriplegia/physiopathology
Chemical Substances	ALS2 protein, human ; Guanine Nucleotide Exchange Factors
Language	English
Publishing date	2008-10-27
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1329381-3
ISSN	2047-783X ; 0949-2321
ISSN (online)	2047-783X
ISSN	0949-2321
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Article: Differenzialdiagnostik der spastischen Spinalparalyse - Abgrenzung neu rodegenerativer Erkrankungen gegen symptomatische Genese

Kassubek, J. / Sperfeld, A.-D.

Psychoneuro

2007 Volume 33, Issue 11, Page(s) 467

Language	German
Document type	Article
ZDB-ID	2102993-3
ISSN	1611-9991 ; 0340-7845
Database	Current Contents Medicine

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Article: Central-core-Myopathie: Eine Erkrankung mit Relevanz im Kindes- und Erwachsenenalter.

Gdynia, H J / Sperfeld, A-D / Hanemann, C O

Der Nervenarzt

2007 Volume 78, Issue 4, Page(s) 387–392

Abstract: Central core myopathy is a nonprogressive or only slowly progressive congenital muscle disease. In most cases, symptoms begin in childhood, but rare cases with adult onset are described. Regardless of its high variability, the clinical hallmarks are ... ...

Title translation	Central core myopathy: a juvenile and adult disease.
Abstract	Central core myopathy is a nonprogressive or only slowly progressive congenital muscle disease. In most cases, symptoms begin in childhood, but rare cases with adult onset are described. Regardless of its high variability, the clinical hallmarks are diffuse muscle weakness and the development of multiple bone deformities and contractures. Skeletal muscle biopsy is of high diagnostic significance. Due to a potential association with malignant hyperthermia, early diagnosis is of great importance. A curative treatment is not currently known. Here we discuss aetiology, pathogenesis, clinical features, diagnosis, differential diagnosis, therapeutic strategies, and prognosis of central core myopathy based on a clinical example with an atypical onset of symptoms in adulthood.
MeSH term(s)	Abnormalities, Multiple/diagnosis ; Adolescent ; Adult ; Bone and Bones/abnormalities ; Child ; Child, Preschool ; Humans ; Infant ; Infant, Newborn ; Muscle Weakness/diagnosis ; Muscle Weakness/therapy ; Myopathy, Central Core/diagnosis ; Myopathy, Central Core/therapy
Language	German
Publishing date	2007-04
Publishing country	Germany
Document type	English Abstract ; Journal Article ; Review
ZDB-ID	123291-5
ISSN	1433-0407 ; 0028-2804
ISSN (online)	1433-0407
ISSN	0028-2804
DOI	10.1007/s00115-006-2237-1
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Zs.MO 446: Show issues

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Article: Neuronale Zeroidlipofuszinosen des Erwachsenenalters.

Gdynia, H J / Sperfeld, A D / Ludolph, A C

Der Nervenarzt

2007 Volume 78, Issue 2, Page(s) 139–40, 142–4

Abstract: Neuronal ceroid lipofuscinoses are a heterogenous group of genetic progressive neurodegenerative disorders. Curative therapeutic strategies are not known. These are largely diseases of childhood; adult-onset forms are rare and poorly characterized. The ... ...

Title translation	Adult-onset neuronal ceroid lipofuscinosis.
Abstract	Neuronal ceroid lipofuscinoses are a heterogenous group of genetic progressive neurodegenerative disorders. Curative therapeutic strategies are not known. These are largely diseases of childhood; adult-onset forms are rare and poorly characterized. The classical adult variant is CLN4 (Kufs' disease), in which autosomal-recessive and autosomal dominant forms are known. Furthermore the "classic infantile" CLN1, caused by a deficiency of the enzyme palmitoylprotein-thioesterase, may be of adult onset Neuronal ceroid lipofuscinoses in adulthood are multifaceted diseases. Their clinical picture is mainly characterized by progressive dementia, seizures, and extrapyramidal motor symptoms. In contrast to the infantile forms, visual loss is an uncommon feature that appears only in adult CLN1 but not CLN4, which may be helpful in clinical differential diagnosis.
MeSH term(s)	Adult ; Dementia/diagnosis ; Diagnosis, Differential ; Female ; Humans ; Male ; Neuronal Ceroid-Lipofuscinoses/classification ; Neuronal Ceroid-Lipofuscinoses/diagnosis ; Seizures/diagnosis ; Vision Disorders/diagnosis
Language	German
Publishing date	2007-02
Publishing country	Germany
Document type	English Abstract ; Journal Article ; Review
ZDB-ID	123291-5
ISSN	1433-0407 ; 0028-2804
ISSN (online)	1433-0407
ISSN	0028-2804
DOI	10.1007/s00115-006-2222-8
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Article: Preclinical Trials – An Update on Translational Research in ALS

Ludolph, Albert C. / Sperfeld, A.-D.

Neurodegenerative Diseases

2006 Volume 2, Issue 3-4, Page(s) 215–219

Institution	Department of Neurology, University of Ulm, Ulm, Germany
Abstract	Translational research has become a strategy which describes the steps from in vitro experimental therapy, its translation into experimental animals and finally to humans. However, this translation of hypotheses from one of these levels to another faces a number of difficulties which are currently of major interest for the development of therapeutics. In particular, in the previously untreatable motor neuron diseases, the steps from genes to transgenic animals and finally to the patient have proven difficult. High expectations have not met with the transfer of numerous therapies from experimental animals to men; there are many in- and outside the field which already question the value of animal models. It is the opinion of the authors that we should not dismiss the models before we have not defined generally accepted standards of protocols for therapeutic studies in experimental animals. Only if a generally agreed standardized and validated methodology in mice cannot predict the human response to therapeutics, an animal model should be abandoned. In contrast, the value of translating genetic findings to experimental animals and men is currently shown for motor proteins; in particular the functional impact of dynactin and dynein for the integrity of motor neurons of rodents and men are in the center of interest.
Keywords	Dynein/dynactin ; Motor neuron disease ; Motor neuron diseases, animal models ; ALS, clinical trials ; ALS, translational research ; Motor neuron diseases, experimental therapies
Language	English
Publishing date	2006-01-06
Publisher	S. Karger AG
Publishing place	Basel, Switzerland
Document type	Article
Note	Therapeutic Strategies
ZDB-ID	2143569-8
ISBN	978-3-8055-8061-8 ; 978-3-318-01307-8 ; 3-8055-8061-4 ; 3-318-01307-2
ISSN	1660-2862 ; 1660-2854
ISSN (online)	1660-2862
ISSN	1660-2854
DOI	10.1159/000089628
Database	Karger publisher's database

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Article ; Online: Myositis associated with localized lipodystrophy

Gdynia H-J / Weydt P / Ernst A / Klein S / Sperfeld A-D / Riecker A

European Journal of Medical Research, Vol 14, Iss 5, p

an unrecognized condition?

2009 Volume 228

Abstract: Abstract Lipodystrophies represent a heterogeneous group of diseases characterized by altered body fat repartition and often metabolic alterations. Here we illustrate a 20 year old male with myositis in association with localized lipodystrophy. ... ...

Abstract	Abstract Lipodystrophies represent a heterogeneous group of diseases characterized by altered body fat repartition and often metabolic alterations. Here we illustrate a 20 year old male with myositis in association with localized lipodystrophy. Immunohistochemical stainings revealed a regular pattern of dystrophin, dysferlin, sarcoglycans, and theletonin. Furtermore, there was no evidence of Lamin A/C deficiency. A nearly identical clinical and histological picture has been described in three patients up to now. Although it is difficult to speculate on a causative pathophysiological mechanism at this time, it is possible that this association represents an unrecognized condition.
Keywords	Lipodystrophy ; lipoatrophy ; myositis ; myopathy ; muscle biopsy ; Medicine ; R
Language	English
Publishing date	2009-05-01T00:00:00Z
Publisher	BMC
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Conference proceedings: Charakterisierung von Patienten mit unkomplizierter und komplizierter Hereditärer Spastischer Paraparese: eine klinische, elektrophysiologische und 3D-MRT-morphometrische Studie

Kassubek, J / Sperfeld, A.D

Aktuelle Neurologie

2005

Abstract: Die Hereditären Spastischen Paraparesen (HSP) stellen eine Erkrankungsgruppe von unterschiedlichem, nur z.T. bekanntem Genotyp und klinisch heterogenem Phänotyp dar, die durch das Leitsymptom einer progredienten Paraspastik und ein Spektrum fakultativer ... ...

Event/congress	78. Kongress der Deutschen Gesellschaft für Neurologie, Wiesbaden, 2005
Abstract	Die Hereditären Spastischen Paraparesen (HSP) stellen eine Erkrankungsgruppe von unterschiedlichem, nur z.T. bekanntem Genotyp und klinisch heterogenem Phänotyp dar, die durch das Leitsymptom einer progredienten Paraspastik und ein Spektrum fakultativer Symptome gekennzeichnet ist. Entsprechend dem klinischen Erscheinungsbild lassen sie sich in unkomplizierte (pHSP) und komplizierte (cHSP) Formen unterteilen. In der vorliegenden Studie wurden 33 HSP-Patienten mit pHSP (n=22) bzw. cHSP (n=11) mittels klinischer, neuropsychologischer und elektrophysiologischer Untersuchungen sowie mittels Magnetresonanztomographie (MRT) untersucht. Das MRT-Standardprotokoll wurde für das Gehirn (einschließlich eines 3D-Volumendatensatzes) und für das obere Rückenmark akquiriert. Neben visuellen Auswertungen der zerebralen MRT und Messungen des Rückenmarksquerschnitts auf verschiedenen Höhen wurden die zerebralen 3D-MRT unter Verwendung der Untersucher-unabhängigen Ganzhirn-basierten Technik der Voxel-basierten Morphometrie (VBM) analysiert, sowohl im Vergleich zu einem gematchten Kontrollkollektiv von Gesunden als auch im Vergleich pHSP zu cHSP. Daneben wurde das Ausmaß der globalen Hirnatrophie standardisiert mittels Brain Parenchymal Fraction-Methode erhoben. Weiterhin wurden Korrelationsanalysen zwischen quantitativen Daten der Neuropsychologie und Elektrophysiologie sowie klinischen und MRT-Parametern berechnet. Mithilfe der elektrophysiologischen Daten konnte das Ausmaß der Affektion des zentralen und peripheren Nervensystems erfasst werden. Trotz der Heterogenität der cHSP zeigte die standardisierte MRT-Analyse zum einen charakteristische Muster in den zerebralen Veränderungen (insbesondere die Weiße Substanz betreffend), zum anderen demonstrierte die VBM-Analyse regionale Läsionsmuster (Atrophie) der Grauen und Weißen Substanz bei beiden Subtypen mit den robustesten Veränderungen im Bereich des Corpus callosum. Mit den Korrelationsanalysen konnten definierte klinische und zusatzdiagnostische Merkmale miteinander und mit einzelnen Bildgebungsparametern in Zusammenhang gebracht werden. Unsere Ergebnisse stellten die unterschiedliche Beteiligung der extrapyramidalen zerebralen und der extrazerebralen ZNS-Strukturen in einer großen Gruppe von pHSP/cHSP-Patienten dar und geben neue Einblicke in pathophysiologische Grundlagen und somit in das Verständnis der Charakteristika dieses heterogenen Krankheitsbildes.
Language	German
Publishing date	2005-10-26
Publishing place	Stuttgart ; New York
Document type	Article ; Conference proceedings
ZDB-ID	124980-0
ISSN	1438-9428 ; 0302-4350 ; 1431-4886
ISSN (online)	1438-9428
ISSN	0302-4350 ; 1431-4886
DOI	10.1055/s-2005-919253
Database	Thieme publisher's database

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