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  • 1
    Electronic Resource
    Electronic Resource
    Epidural anaesthesia and spinal arachnoiditis (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Anaesthesia 44 (1989), S. 0 
    Details
    ISSN: 1365-2044
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Six patients were referred to our hospital with spinal arachnoiditis after epidural anaesthesia performed one month to 3 years before the onset of symptoms. None had had previous lumbar surgery or trauma, intraspinal haemorrhage, infections or other known causative factors of arachnoiditis. All the patients were free of neurological symptoms before epidural anaesthesia and only two had transient distress in the period immediately following the procedure. The clinical signs and symptoms of spinal arachnoiditis were severe and in every case the diagnosis was confirmed by myelography. Three patients were confined to a wheelchair after 3 years of follow-up. To our knowledge, the anaesthetic procedures were performed according to standard methods. Arachnoiditis seems to be due to the epidural injection of foreign substances, and may be related to anaesthetic–vasoconstrictor solution or contaminants.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2044.1989.tb11285.x
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  • 2
    Electronic Resource
    Electronic Resource
    Homozygous hypertrophic hereditary motor and sensory neuropathies (1994)
    Springer
    Neurological sciences 15 (1994), S. 5-14 
    Details
    ISSN: 1590-3478
    Keywords: hereditary motor and sensory neuropathy ; Charcot-Marie-Tooth disease ; Dejerine-Sottas disease ; demyelination ; hypomyelination ; 17p11.2 duplication
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Abbiamo paragonato 25 pazienti affetti da neuropatia ereditaria sensitivo-motoria (HMSN) di tipo I ad ereditarietà autosomica dominante con 7 pazienti affetti da neuropatia ipertrofica ad ereditarietà non dominante. Tutti i pazienti con ereditarietà autosomica dominante sono risultati portatori della duplicazione 17p11.2, dimostrando così che questa è largamente presente nelle famiglie HMSN I. Il secondo gruppo comprendeva: due fratelli affetti da una neuropatia ipertrofica di gravità nettamente diversa, nati da genitori sani non consanguinei; due sorelle con un fenotipo HMSN I nate da genitori sani, cugini di primo grado; due fratelli con un fenotipo HMSN III nati da genitori non consanguinei affetti da HMSN II; un bimbo con un classico fenotipo HMSN III, nato da genitori sani, non consanguinei. L'analisi del DNA ha dimostrato l'assenza della duplicazione sia nei pazienti del secondo gruppo che nei loro genitori. I nostri dati dimostrano che: l'HMSN III è eterogenea e comprende pazienti omozigoti per differenti geni neuropatogeni; è opportuno tenere distinti i pazienti con neuropatia ipertrofica recessiva da quelli portatori di una HMSN Ia dominante poiché è probabile che le due malattie siano dovute a difetti genici diversi.
    Notes: Abstract We compared 25 autosomal dominant hereditary motor and sensory neuropathy (HMSN) type I patients with 7 subjects affected by hypertrophic HMSN with non-dominant inheritance. All the autosomal dominant HMSN I cases carried the chromosome 17p11.2 duplication, providing evidence that it is widely represented in HMSN I families. The second group included: Two siblings born to unrelated, unaffected parents and suffering from hypertrophic HMSN of strikingly different severity; two sisters with HMSN I phenotype, born to first-cousin unaffected parents; two brothers with HMSN III phenotype born to unrelated parents both showing HMSN II phenotype; a child with classic HMSN III phenotype, born to unrelated, unaffected parents. The 17p11.2 duplication was not found in any of the patients of the second series or in their parents. Our data provide further evidence that: HMSN III is heterogeneous and encompasses the homozygous expressions of different neuropathic genes; it is advisable to separate autosomal recessive hypertrophic HMSN from dominant HMSN Ia, because they appear to be due to different DNA mutations.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02343492
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  • 3
    Electronic Resource
    Electronic Resource
    Neurogenic muscle hypertrophy (1989)
    Springer
    Journal of neurology 236 (1989), S. 292-295 
    Details
    ISSN: 1432-1459
    Keywords: Muscle hypertrophy ; Neuropathies ; Spontaneous muscular activity ; Stretching
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Muscle hypertrophy is rare in denervating diseases. A patient with calf enlargement associated with L5–S1 radiculopathy and another with thenar, hypothenar, forearm and calf muscle hypertrophy in the course of chronic relapsing inflammatory demyelinating polyneuropathy are described. Gastrocnemius muscle biopsy revealed both type I and type II fibre hypertrophy in the former case and predominant type I fibre hypertrophy in the latter. Passive stretching and abnormal spontaneous muscular activity might have played a role in the origin of hypertrophy in both patients, but a satisfactory explanation for denervation hypertrophy has yet to be provided.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00314459
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  • 4
    Electronic Resource
    Electronic Resource
    Recurrent familial neuropathy due to liability to pressure palsies (1988)
    Springer
    Neurological sciences 9 (1988), S. 355-363 
    Details
    ISSN: 1590-3478
    Keywords: Hereditary neuropathy ; pressure palsies ; Schwann cell dysfunction ; myelin abnormalities
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Due famiglie portatrici di neuropatia ereditaria con predisposizione alla paralisi da compressione (HNPP) sono state valutate dal punto di vista clinico, neurofisiologico e morfologico (2 casi indice). L'eredità in entrambi i casi è autosomica dominante. La presentazione clinica è caratterizzata da mononeuropatie non dolorose, transitorie e ricorrenti, in distretti diversi, precipitate da compressioni o traumi lievi, o senza causa apparente. Nei soggetti più anziani in particolare è presente un quadro clinico di lieve polineuropatia sensitivomotoria. Nei membri affetti le velocità di conduzione del nervo sono rallentate in modo diffuso, con blocchi focali nei comuni siti di intrappolamento. L'esame dei potenziali evocati somatosensitivi ha dimostrato in tutti i soggetti affetti anomalie di tipo periferico, coinvolgenti anche i tratti prossimali in modo più pronunciato nei pazienti anziani. All'esame della singola fibra nervosa isolata, rigonfiamenti della guaina mielinica con l'aspetto caratteristico dei “sausages” sono riscontrabili nel 40 e nel 75% delle fibre esaminate rispettivamente nei 2 casi indice. All'esame ultrastrutturale i “sausages” appaiono formati da un eccessivo numero di lamelle mieliniche, alcune formanti loops, che sembrano costringere l'assone.
    Notes: Abstract Two families with hereditary neuropathy with liability to pressure palsies (HNPP) were evaluated clinically, electrophysiologically and pathologically (2 index cases). Familial patterns suggest autosomal dominant inheritance. The clinical presentation is characteristic, with recurrent painless transitory mononeuropathy, without evident triggering factors, or caused by trivial trauma or pressure. In affected members neurophysiological studies showed diffuse slowing in nerve conduction, more evident at common sites of entrapment. Somatosensory evoked potentials (SEPs) showed bilateral peripheral abnormalities with proximal nerve incolvement more pronounced in older patients. 40 to 75% of teased fibers displayed sausage-shaped swellings. Ultrastructurally some sausages showed redundant myelin loops and excessive number of myelin lamellae that seem to cause axonal constriction.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02333999
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  • 5
    Electronic Resource
    Electronic Resource
    Hereditary motor and sensory neuropathy type I and type II (1990)
    Springer
    Neurological sciences 11 (1990), S. 471-479 
    Details
    ISSN: 1590-3478
    Keywords: Neuropathy ; hereditary motor and sensory neuropathy ; Charcot-Marie-Tooth disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Allo scopo di distinguere chiaramente in sottogruppi i pazienti affetti da HMSN, abbiamo studiato prospeticamente dal punto di vista clinico, genetico ed elettrofisiologico 128 soggetti: 46 casi indice, 39 parenti affetti e 43 sani. La diagnosi di HMSN I o II è state posta in 77 casi. La distinzione tra I e II tipo è risultata impossibile dal punto di vista clinico, ma lo studio delle velocità di conduzione ha dimostrato una chiara divisione in due popolazioni. Il comportamento delle VCM è risultato omogeneo all'interno delle singole famiglie. L'ereditarietà, autosomica dominante in quasi tutti i casi, è risultata probabilmente recessiva in tre soggetti con HMSN I, mentre l'analisi dell'albero genealogico suggerisce una trasmissione X-linked in un'altra famiglia di I tipo. Non abbiamo trovato linkage con il locus Duffy. In queste neuropatie fenotipi simili possono essere determinati da differenti difetti genici. La presenza di un diffuso rallentamento della conduzione nervosa periferica, evidenziata dal confronto tra velocità di conduzione della risposta Fe VCM del nervo ulnare depone per una patogenesi primitivamente mielinica dell'HMSN I.
    Notes: Abstract In an attempt to clearly identify the different HMSN subgroups, we prospectively evaluated 128 subjects (46 index cases, 39 affected and 43 unaffected relatives) on clinical, genetic and electrophysiological grounds. The diagnosis of HMNS I or II was made in 77 patients. Differential diagnosis between type I and II patients was impossible on clinical grounds alone, but nerve conduction study showed a clearcut subdivision into two populations. MCV behavior was consistent within families. Inheritance, autosomal dominant in almost all cases, was probably recessive in three HMSN I subjects and pedigree analysis pointed to X-linked transmission in one HMSN I family. We found no evidence for linkage to Duffy locus. We think that similar HMSN phenotypes can be determined by different gene defects. Ulnar nerve F-conduction velocity did not significantly differ from distal MCV in HMSN I: the evidence of a diffuse slowing of nerve conduction supports the hypothesis of a primary myelin defect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02336567
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  • 6
    Electronic Resource
    Electronic Resource
    The POEMS syndrome: Report of six cases (1994)
    Springer
    Neurological sciences 15 (1994), S. 353-358 
    Details
    ISSN: 1590-3478
    Keywords: polyneuropathy ; POEMS syndrome ; monoclonal gammopathy ; osteosclerotic myeloma ; Castleman's disease ; cytokines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Descriviamo 6 pazienti con sindrome POEMS (Polineuropatia, Organomegalia, Endocrinopatia, gammopatia Monoclonale, alterazioni cutanee [Skin changes]), una peculiare malattia ad interessamento multisistemico frequentemente associata a mieloma osteosclerotico o ad altre malattie plasmacellulari. Una polineuropatia sensitivo-motoria si accompagnava ad interessamento pluriviscerale in tutti i pazienti, a mieloma osteosclerotico in due casi, a gammopatia monoclonale di incerto significato in altri due, e a malattia di Castleman negli ultimi due pazienti. In tutti i 6 casi la biopsia del nervo surale dimostrava una neuropatia mista, demielinizzante ed assonale. In due casi sono stati trovati elevati livelli di Interleuchina-6, ma la patogenesi della malattia è ancora ignota.
    Notes: Abstract We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02339932
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  • 7
    Electronic Resource
    Electronic Resource
    Immunological and endocrinological abnormalities in paraneoplastic disorders with involvement of the autonomic nervous system (1997)
    Springer
    Neurological sciences 18 (1997), S. 157-161 
    Details
    ISSN: 1590-3478
    Keywords: Autonomic dysfunctions ; Paraneoplastic syndromes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Descriviamo quattro casi con una sintomatologia d'esordio di una neoplasia sistemica caratterizzata da sintomi disautonomici ed una complicanza neurologica non metastatica a patogenesi autoimmune ed endocrina. La serie include: un paziente con leucemia acuta e poliradiculoneuropatia 〉 autonomica e sensitivo-motoria, un paziente con carcinoma del colon, neuropatia autonomica ed encefalite limbica, un paziente con neoplasia polmonare, neuropatia autonomica ed encefalopatia ipercalcemica, infine, un paziente con carcinoma a piccole cellule associato a neuropatia autonomica in sindrome miastenica di Lambert-Eaton (LEMS) e sindrome da inappropriata secrezione di ormone antidiuretico (SIADH). Sottolineiamo l'importanza prognostica della disfunzione autonomica, frequentemente associata ad altre sindromi neurologiche paraneoplastiche, e ne discutiamo il possibile ruolo nelle concomitanti complicanze ad origine autoimmune e/o disendocrina.
    Notes: Abstract We report a series of four patients in whom the onset of systemic cancer was heralded by dysautonomic symptoms and a neurological non-metastatic complication mediated by immunological and endocrine factors. The series includes: a patient with acute leukaemia and autonomic sensory-motor polyradiculoneuropathy, a patient affected by colon carcinoma and autonomic neuropathy and limbic encephalitis, a patient with lung cancer and autonomic neuropathy and hypercalcaemic encephalopathy, a patient with small cell lung cancer associated with autonomic neuropathy in Lambert-Eaton Myasthenic Syndrome (LEMS) and syndrome of inappropriate ADH secretion (SIADH). We underline the prognostic importance and discuss the possible etiopathogenetic role of autonomic dysfunction, which is frequently associated with paraneoplastic neurologic syndromes of autoimmune and/or dysendocrine origin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02048484
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