Biomedical subjects
Publications and source records attributed to R Escourolle.
The main technics of peripheral nerve biopsy examination, the diagnostic value of each of them and their limits are given. Chief pathological processes and changes and usual causes of peripheral neuropathies, the diagnosis of which can be provided by nerve biopsy are analysed. Most frequent errors and misleading changes are emphasized. An examination scheme is set up.
Two cases of thalamic lesions with motor neglect are presented. The syndrome of motor neglect was complete in those cases with a) underutilization of left limbs, but good utilization upon verbal orders, b) loss of placement reaction, c) weakness of movement when hand was approaching the target, d) weakness of motor reaction to nociceptive stimuli. Those cases confirm that motor neglect exists after thalamic lesions and bring pathologic clues for topographic discussion. Motor neglect seems to be a particular case of partial unilateral neglect throwing some doubt on the hypothesis of a global trouble of hemispheric activation. Prevalence of left motor neglects suggests some linkage between propositional motility and language. One may suppose that in the right hemisphere language is able to have a vicarious action when spontaneous activation is lost; at the opposite, in the left hemisphere language and motility would be too linked to let this dissociation be generally possible.
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The concept of cerebellar atrophy was first introduced by Pierre Marie in 1893 in his description of hereditary cerebellar ataxia. Subsequent criticism of this notion was refuted by the report of new clinicopathological entities which are compatible with it. The contributions of the Clinic for Diseases of the Nervous System are discussed in this paper. Etiological classification is difficult, in spite of progress made in the genetic, epidemiologic, and biochemical approaches to cerebellar atrophies. Pathologic findings appear to supply the most secure basis for presentation of these affections. Predominantly cortical atrophies may be localized to the vermis. This is the case in the familial cerebello-olivary atrophy of Holmes (1907), in tardive cortical atrophy (Pierre Marie, Foix and Alajouanine, 1922), and in the cerebellar atrophy of alcoholics (Alajouanine, Castaigne, Contamin and Lebourges, 1959; Victor, Adams and Mancall, 1959), in which the lesions are similar. The frequent intrication of the various etiological factors: age, deficiencies, alcohol, heredity, suggests the role of a sometimes primary, sometimes latent genetic predisposition, revealed during various pathological conditions. Cortical cerebellar atrophy may be of the diffuse type, as in paraneoplastic cerebellar atrophy (Brouwer and Biemond, 1938), which is closely related to subacute polioencephalomyelitis in cancer patients (Dubas et al., 1982), and in congenital atrophy of the granular layer (Norman, 1940), which is more a dysgenesis than a true degenerative affection. Lesions affecting mainly the efferent or afferent cerebellar pathways include olivopontocerebellar atrophy (Dejerine and André Thomas, 1900) which should be included in the larger overall concept of multiple system atrophy (Oppenheimer, 1976), and dentorubric atrophy (Ramsay Hunt, 1921) which themselves should be integrated in the group of spinocerebellar atrophies. Finally, olivorubrocerebellar atrophy (Lejonne and Lhermitte, 1909) and crossed cerebellar atrophy, traditionally studied together with cerebellar atrophies, are only the result of a pre-existing lesion.
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Two anatomo-clinical cases of downward gaze palsy and one case of upward gaze palsy are reported. A tonic and intermittent downward gaze deviation is described. The supranuclear palsies of the downward gaze were related to paramedian lesions of the rostral mesencephalon; the lesions involved the rostral interstitial nucleus of the medial longitudinal fasciculus, the nucleus interstitial of Cajal, and/or their afferent and/or efferent pathways. The supranuclear palsy of the upward gaze was related to lesions of the posterior commissure. Tonic and intermittent downward deviation of gaze and ocular bobbing have opposed features. The former could be related to disinhibited reticular mesencephalic neurones activated by vestibular inputs. Tonic upward deviation of gaze is also related to a vestibulo-ocular reflex. In this case, partial or total damage of the nucleus of Cajal, and/or its input and/or its output fibers appears to have a critical role.
Two chronic alcoholic patients developed severe encephalopathy while receiving high doses of parenteral thiamine and pyridoxine. Both presented with unusually marked hypertonia of the gegenhalten type, myoclonias and fluctuating mental impairment with memory disturbances and hallucinations. Later on, one patient went into stupor and coma and died ; the other recovered after parenteral niacin treatment. No skin lesions or diarrhoea were observed. Post-mortem examination of the first patient confirmed the presence of a pellagra-like encephalopathy characterized by widespread neuronal chromatolysis. There were no changes suggesting an associated alcoholic encephalopathy.
Brain bovine gangliosides added for 48 hrs. to the culture medium at concentrations ranging from 5.10(-5) mg/ml to 5.10(-7) mg/ml induced a statistically significant (p less than .005 to p less than 000,5) increase of the number of neurites of the outgrowth zone of guinea-pig spinal ganglia. On the contrary, the neurite length was only slightly increased.
We performed a lipid analysis on nerve biopsy specimens in two cases of degenerative hypertrophic neuropathy. Quantitative analysis of the major lipid classes, ie, cholesterol, cerebrosides, sulfatides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine, phosphatidyl-inositol, sphingomyelin, and gangliosides, were performed. The two cases exhibited extreme decreases in levels of lipids that could be related to the very low myelin content of these nerves. Cholesterol and phospholipid levels were especially reduced. Cerebrosides and sulfatides were not modified in the same proportion, as could have been predicted from the degree of demyelination. This relative glycolipid increase could be due to the very high Schwann cell proliferation.
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The clinical and neuropathological findings in 28 cases of paramedian thalamic and midbrain infarcts are reported. The 4 instances of unilateral paramedian thalamic infarct were characterized by mood and behavioral changes, limitation of the infarct to the center of the anatomical paramedian territory, and symmetrical configuration of the paramedian thalamic arteries. Basilar artery occlusion was found in 1 patient. The 5 cases of bilateral paramedian thalamic infarcts were characterized by disturbances of consciousness and behavior, extension of the infarct (to the mammillothalamic tracts in 4 cases, the red nuclei in 3, and the hypothalamus in 2), and a variable paramedian thalamic arterial pattern. The arterial pattern was symmetrical in 2 cases, asymmetrical in 1, and unilateral in 1. The basilar artery was occluded in 1 case, the basilar communicating and posterior cerebral arteries in 1, and a third patient had occlusion involving an aneurysm of the basilar artery. The 19 patients with paramedian thalamopeduncular infarcts had marked disturbances of consciousness (hypersomnia, deep coma, akinetic mutism) associated with ocular motility changes. Later, abnormal movements--always delayed--and memory disturbances were observed in some. Thalamic changes were restricted to beh paramedian territory in only 3 cases. The arterial pattern was symmetrical in 5. The basilar and posterior cerebral arteries were occluded in 4 patients each. Paramedian infarcts were rarely found as isolated lesions and were always bilateral when there was only one arterial pedicle. The paramedian thalamic pedicle can supply the polar thalamic territory.
Cerebellar changes have been found in 41/50 cases of Creutzfeldt-Jakob disease. They were severe in 9 cases. We did not find significant correlation between the cerebellar symptoms and signs pointed out in clinical records and prominent cerebellar changes. The only exception consisted in dentate nucleus involvement which was more frequently related to those symptoms and signs. 5 of the cases with severe changes were characterized by predominant granule cell atrophy without kuru plaques. The mean age of death (56.0) was significantly lower in this variant than that of patients with other cerebellar changes (64.4) (p less than 0.01). The granule cell atrophy seems a distinct variant on the basis of age of death and pathological changes. However, it is not characterized by the presence of kuru plaques.
Clinical and neuropathological studies of a case of pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis (ALS) in a young woman with a strong likelihood of a similar familial past medical history have been presented. Microscopic examination revealed neuronal loss and gliosis of globus pallidus, corpus luysii and substantia nigra. Pallor of the pyramidal tracts and neuronal loss in hypoglossal nuclei and anterior horns with gliosis were present. The rarity of the association of a pallido-luyso-nigral atrophy and an ALS, the occurrence of an ALS at such a young age and the fact that her grandmother died of Parkinson disease at age 30 suggest that this association may represent more than a coincidental occurrence.
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Cases of associated angio-immunoblastic lymphadenopathy (AIL) and peripheral neuropathies have been rarely reported in the literature. Three such cases are described, in two men and one woman aged 79, 59, and 45 years respectively. Diagnosis of AIL was confirmed by lymph node biopsy in the two latter patients during their lifetime, and in the first case from examination of cervical nodes at autopsy. All three patients presented neurological disorders, of the polyradiculoneuritis type in the first case, multiple neuritis of the lower limbs followed by radiculalgia in the second, and myalgia and neuralgia with neurogenic signs in the EMG in the third case. No evidence of a toxic, metabolic, or infections aetiology was found, histological examination of nerve and muscle specimens demonstrated localised AIL lesions in one case, and discrete lymphoplasmocytic infiltration of the peripheral nervous system in the other two patients.
Four different strains of Creutzfeldt-Jakob disease virus (2 primary and 2 passaged in primates or mice) were inoculated intra-cerebrally into squirrel monkeys implanted with continuously-recording indwelling electrodes. Simultaneous EEC and videotape recordings were made on unrestrained animals. In addition EEG recordings were made of evoked visual potentials on restrained animals. EEG abnormalities appeared in every animal before the first clinical signs (6 to 20 months after inoculation) and included generalized slowing, epileptiform patterns and occasional episodes of pseudo-periodic activity. Abnormal evoked visual potentials and disturbances of consciousness were also noted. All viral strains produced similar disorders and the death of inoculated animals. The relative frequency of epilepsy seen in the CJD-inoculated squirrel monkey contrasts with its irregular occurrence in most other monkey species, and its total absence in the spider monkey. This could be related to the lesser complexity of neo-cortical evolution in the squirrel monkey and a less pronounced development of inhibitory CNS mechanisms under the general control of GABA-ergic neurons.
Two anatomo-clinical cases of a necrotic form of demyelinating disease are reported. The disease occurred in two women, had a late onset (patient were about 50 years old) and had a relapsing-remitting course during more than 10 years. The CSF displayed a high protein level over 125 mg/100 ml whereas the gamma-globulin level was normal. The anatomical study found symmetrical cavitations involving both hemispheres and optic tracts with clear-cut limits. Axons and myelin were both destroyed, only the vascular network being partially spared. At the lesion's border-line mononuclear cell infiltrates as well as some phagocytes with sudanophilic inclusions were found. The scarcity of the compound granular corpuscules suggest an old pathological process. A narrow zone of myelin-axonal dissociation was also observed. Astrocytic proliferations was unimportant. Blood vessels were normal. In one case plaques of multiple sclerosis were found in the spinal cord. Those two cases are unusual forms of a diffuse disseminated sclerosis: multiple sclerosis and Schilder's disease are considered as two anatomo-clinical variants of the same pathological process. The observed necrotic lesions are different from the acute necrotic forms of multiple sclerosis as the latter have rapidly developed. The long lasting course of the disease, over 10 years, allowed a complete resolution of the lesions explaining the cavitations. The late onset of the disease and the CSF high protein level are pointed out. The significant of the high protein level and normal gammaglobulin level in the CSF is discussed.
Various questions are asked to the neuropathologist when the diagnosis of malignant glial tumor is suspected: is there a tumor on the sample? is it a glial tumor? What is the degree of malignancy and the prognosis? Answers to be given are easier if the biopsy has been performed before any treatment than in case of suspicion of tumor recurrence. The limits of the neuropathological methods, as well classical as more up to date ones such as electron microscopy, immunohistochemistry, image analysis or tissue culture and those of other research methods such as biochemistry, are emphasized.