Klinischer Bericht über den siebenten Fall von spastischer Paraplegie in einer Familie und Ergebnis der dritten Autopsie aus derselben Familie

Newmark, Leonard

doi:10.1007/bf01629416

Cited by 12 publications

(4 citation statements)

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“…Accounts of the axonal pathology observed in the long tracts of the spinal cord characteristic of HSP have been documented for over a century [10,11]. However, as the disease rarely shortens the life span of its patients and only a small fraction of these patients die in hospital, the number of neuropathological studies reported in the literature is sparse [7,8,[12][13][14][15][16][17][18][19][20][21][22][23][24][25]. As a result, descriptions of the distribution and extent of axonal changes that occur in the corticospinal and sensory tracts, at post-mortem, have been qualitative.…”

Section: Discussionmentioning

confidence: 99%

The extent of axonal loss in the long tracts in hereditary spastic paraplegia

DeLuca

Ebers

Esiri

2004

Neuropathology Appl Neurobio

170

115

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Hereditary spastic paraplegia (HSP) comprises a group of inherited neurodegenerative disorders with the shared characteristics of progressive weakness and spasticity predominantly affecting the lower limbs. Limited pathological accounts have described a 'dying back' axonal degeneration in this disease. However, the distribution and extent of axonal loss has not been elucidated in a quantitative way. We have studied post-mortem material from six HSP patients and 32 controls in detail. The population of axons was examined quantitatively in the corticospinal tracts from the medulla to the lumbar spinal cord and the sensory tracts from the lumbar to upper cervical spinal cord. Myelin and axon-stained sections were employed to estimate the notional area and axonal density, respectively, of both tracts. Our results indicate that in the corticospinal tracts there is a significant reduction in area and axonal density at all levels investigated in HSP compared to controls. In the corticospinal tracts, the ratio of medulla and lumbar total axonal number was significantly greater in HSP cases compared to controls suggesting more pronounced axonal loss in the distal neuraxis in HSP than in controls. The sensory tracts in HSP, in contrast, showed a significant reduction in area and axonal density only in the upper regions of the spinal cord. Similar to the corticospinal tracts, the ratio of lumbar and upper cervical cord total axonal number in the sensory tracts was increased in HSP cases compared to controls. These findings are consistent with a length-dependent 'dying back' axonopathy. Nerve fibre loss was not size-selective with both small and large diameter fibres affected. In HSP, axonal loss is widespread and symmetrical and its extent tract-specific. The characterization of the nature of axonal loss in HSP, where this is a primary phenomenon, may help the interpretation of axonal loss in conditions such as multiple sclerosis where the sequence of events is less clear.

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Section: Discussionmentioning

confidence: 99%

The extent of axonal loss in the long tracts in hereditary spastic paraplegia

DeLuca

Ebers

Esiri

2004

Neuropathology Appl Neurobio

170

115

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“…Newmark [14] described a patient with FSP whose walking deteriorated appreciably during an attack of typhomalarial fever. The neurological symptoms may worsen during attacks of fe-Ver in Friedreich disease [1].…”

Section: Discussionmentioning

confidence: 99%

Familial spastic paraplegia with peroneal amyotrophy. A family with hypersensitivity to pyrexia

Serena¹,

Rizzuto

Moretto

et al. 1990

Ital J Neuro Sci

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We describe 4 siblings with spastic paraparesis and peroneal amyotrophy who were prone to severe pain and painful dysesthesias, tetraparesis and pyramidal signs during pyrexial episodes of variable etiology. These symptoms cleared almost completely in 10-20 days. Nerve conduction velocity was reduced more markedly during the spells of fever. Muscle biopsy specimen was normal. Some transient functional disturbance of membrane equilibrium of the nervous pathways of both central and peripheral nervous systems was probably responsible for the attacks during pyrexial episodes.

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“…Since then, the disease has been understood as a distal axonopathy of the longest large myelinated fibers of the spinal tract. In 1952, Schwarz 37 gave an extensive review of the pathologic literature, highlighting the contributions of Newmark (1904Newmark ( , 1906Newmark ( , 1911 …”

Section: Structural Characterization: Neuropathology and Neuroimagingmentioning

confidence: 99%