Previous and recent studies stress the importance of small intracerebral vessel lesions in the pathogeny of some forms of dementia. The observations on this subject and included in our study refer to adult patients (32-86 years) selected only on the criterion of clinical diagnosis of dementia. To diagnose most of the cases, clinical experimental data and clinico-psychomotor tests were used. Classical neuropathological techniques were performed for the morphological study of their brains. All types of cerebral vessels were affected in various percentages by the different types of lesions. Vessel wall sclerosis of all types of intracerebral vessels was noticed in 50% of the cases, the thickening of the arteriolar wall with or without sclerosis in 25% of cases and exclusively capillary fibrosis in 21.4% of cases. Other types of vascular changes were present in a small number of cases. No correlation could be made between a certain type of vascular lesion and the age or diagnosis of the patient. Nevertheless, we could observe that wall sclerosis was more frequently found in groups with the oldest patients with vascular diseases (VD, MD), the thickening of the arteriolar wall in patients aged 60-74 years with vascular diseases, and capillary changes in older patients with dementia of Alzheimer type. No preferential location of the vascular lesions could be observed in our group of patients.

Adult↗

PubMedDate not supplied

Chronic sensorimotor polyneuropathy associated with cerebello-extrapyramidal manifestations in a patient with tomaculous neuropathy.

Clinical, electrophysiological and morphological (sural nerve and gastrocnemius muscle biopsies) data of a 57-year-old man with a chronic sensorimotor polyneuropathy of Charcot-Marie-Tooth type associated with a progressive cerebello-extrapyramidal syndrome are reported. Patient's family data were negative. Nerve structural and ultrastructural examinations revealed the morphological picture of a tomaculous neuropathy. The association of different clinical syndromes and the specificity of the tomaculous neuropathy are discussed.

Basal Ganglia Diseases↗

PubMedDate not supplied

Degenerative neuromuscular diseases with a late onset. Clinical, electrophysiological and morphological study.

This study carried out clinical, electrophysiological and morphological investigations (sural nerve and gastrocnemius muscle biopsies) in a group of 47 patients with neuromuscular disease of a certain or supposed degenerative origin and a late onset (over the age of 30 yrs.). It aimed the evidence of the eventual particularities of such diseases with a delayed onset. The equal involvement of sexes, the insidious onset, the clinical picture similar to that of the corresponding diseases with an onset at the usual age were observed. Regardless of the age, some interesting associations of the polyneuropathy with other diseases or its presence with in these diseases (Parkinsonism, Addison's disease, multiple symmetrical lipomatosis, etc.) were noticed, too. Electrophysiological examinations showed no particularities. Neither did the muscular morphological picture in most of the cases presenting neurogenic lesions with a moderate intensity. The sural nerve biopsy evidenced in 70% of the cases a moderately intense neuropathy of an axonal type ("dying back"), with or without secondary lesions of segmental demyelination and with the signs of a live regenerative activity.

Adult↗

PubMedDate not supplied

Tomaculous neuropathy with unusual clinical aspects.

Tomaculous neuropathy represents the morphological substrate of the recurrent familial neuropathy with liability to pressure palsies. Some ultrastructural changes characterizing the tomaculous neuropathy can occur as incidental aspects in other different neuropathies. Few tomaculous neuropathy cases with clinical aspect of chronic polyneuropathy without paretic episodes have been mentioned in the literature. In the present work, we report four cases who offered the morphological surprise of a true tomaculous neuropathy with 15-37% of the teased fibres bearing tomaculae sized: 55-106 microns/20-23 microns, on the background of a demyelinating neuropathy with 25-56% of the teased fibres showing segmental de- or remyelination. The clinical and electrophysiological diagnoses of these 4 patients were: HSMN type I (2 cases), HSMN type VIII (polyneuropathy associated with a cerebello-extrapyramidal syndrome -1 case), and a neurogenic scapuloperoneal syndrome (1 case). The specificity of the tomaculous neuropathy is discussed.

Adolescent↗

PubMedDate not supplied

Biochemical, histological and electron microscopical study of experimentally induced cortisone myopathy.

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Animals↗

PubMedDate not supplied

Increased intracellular calcium triggered by immune mechanisms in amyotrophic lateral sclerosis.

Although the causes of motor neuron degeneration and death in amyotrophic lateral sclerosis (ALS) is unknown, recent evidence suggests a prominent role for increased intracellular calcium, possibly triggered by autoimmune mechanisms. The presence in ALS patients of paraproteinemias, lymphomas, lymphoid cells in the central nervous system (CNS) and the availability of animal models of immune-mediated motor neuron disease provide circumstantial evidence for autoimmunity. Direct evidence derives from the demonstration that ALS IgGs bind to voltage-gated calcium channels in 75% of sporadic cases, but not in familial ALS cases, and that ALS IgGs increase N-type and P-type calcium currents in neuronal cells and in lipid bilayers. These same ALS IgGs are cytotoxic for a motor neuron cell line (VSC 4.1) in vitro. In addition, following passive transfer to mice in vivo, ALS IgGs produce ultrastructural and calcium changes in synaptic vesicles and mitochondria of motor axon terminals, as well as in rough endoplasmic reticulum and Golgi complex of motor neuron perikarya, but not in sensory neurons or Purkinje cells. The reason for the selective vulnerability of motor neurons is not clearly defined, but a prominent possibility is the physiological absence in motor neurons of the calcium-binding proteins calbindin-D28k and parvalbumin. These studies emphasize the central role of increased intracellular calcium in motor neuron cell death in sporadic ALS, and the role of autoimmunity in triggering such increases.

Amyotrophic Lateral Sclerosis↗

PubMedDate not supplied

A multidisciplinary approach of the psycho-somatic involution syndrome.

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Aged↗