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Biomedical subjects

R Gherardi

Publications and source records attributed to R Gherardi.

At least 145 records · Page 8Linked to original sources

Fatal nonmeningitic Listeria rhombencephalitis. Report of two cases.

Two cases of rapidly fatal Listeria rhombencephalitis with normal cerebrospinal fluid (CSF) findings occurred in previously healthy adults. The infection presented with nausea and headache followed by fever and signs of lower cranial nerve dysfunction, without associated meningismus, and progressed to death within four and six days of hospitalization. Because of normal CSF findings (including ventricular fluid in one patient) and negative culture results of both blood and CSF, the diagnosis was not suspected. Listeriosis should be considered early in any febrile patient presenting with signs of brain-stem dysfunction, even if CSF findings are normal.

Adult↗

[Cerebral lymphoma associated with lesions of multiple sclerosis].

A 24 year-old man experienced a left retrobulbar neuritis which improved completely after 2 months of non-steroid antiinflammatory therapy. One month after the end of the treatment he developed a Korsakoff-like amnestic syndrome. Three months later he complained of horizontal diplopia. A CT Scan showed a diffuse enhancement of the periventricular areas, corpus callosum and fornix. Diplopia and CT scan abnormalities disappeared after the administration of tetracosactide. Subsequently a progressive worsening of the neurological condition developed, including a 1 1/2 syndrome of Fisher. In C.S.F. proteins ranged from 35 to 66 mg/dl, gammaglobulins from 4 to 5 per cent, cells from 2.2 to 6.8 per mm3 without abnormal cells. Rounded areas of enhancement were observed on CT scan in pons and right occipital lobe. Usual biological tests, abdominal echography and lymphography were normal. Death occurred 15 months after the onset of symptoms. Neuropathological examination showed: 1) a cerebral lymphoma of probable B origin with distinct masses in right occipital lobe and pontine tegmentum and a more diffuse perivascular infiltration on the left side in the amygdaloid nucleus, fourth temporal gyrus, sublenticular area, hypothalamus and in the right internal capsule; 2) multiple small clear-cut foci of demyelination with myelin-axonal dissociation bilaterally in the optic pathways, periventricular regions, corona radiata, cerebral and cerebellar white matter, sublenticular areas, temporal lobes, splenium of the corpus callosum and fornices with secondary atrophy of the mamillary bodies. Both recent and old plaques were observed. Inflammatory perivascular cuffing, when present, consisted of small nontumoral lymphocytes.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Asymptomatic encephalic intraparenchymatous neuroepithelial cysts.

The authors report three cases of intraparenchymatous neuroepithelial cysts, which did not communicate with the ventricular system and the subarachnoid space. They were located in (1) the right frontal lobe, (2) the left cerebral peduncle and pons, and (3) the right cerebellar hemisphere. All of them were asymptomatic, despite their volume and location, and presented as incidental autopsy findings. A developmental origin is likely for these non-neoplastic cysts.

Aged↗

Adult form of Leigh's disease: a clinico pathological case with CT scan examination.

The clinical and pathological findings of a 31-year-old woman, in whom the diagnosis of Leigh's disease was made, are reported. CT scan examination with contrast enhancement showed symmetrical areas of low density, in both thalami, anterior limbs of internal capsules and corpus callosum. Longstanding chronic lesions involved the optic chiasma and the cerebral peduncles and consisted of myelin loss, status spongiosus, astrocytic gliosis and marked capillary proliferation. The neurons were spared. In the basal ganglia, internal capsules and corpus callosum, these lesions were more recent and consisted of focal necrosis, perivascular oedema and few lymphocytic perivascular cuffings.

Adult↗

Symptomatic xanthogranuloma of the third ventricle: a clinicopathological report.

The clinical and computerized tomodensitometric data and postmortem examination findings from a case of xanthogranuloma of the third ventricle, producing hydrocephalus, are described. A review of 8 recently reported cases resembling ours shows that, on clinical and radiological grounds, third ventricle xanthogranulomas are usually indistinguishable from colloid cysts. From an embryologic and pathologic point of view, these entities seem also to be closely related.

Aged↗

[Peripheral nervous system and panarteritis nodosa. Review of 27 cases].

Twenty-seven cases of panarteritis nodosa (PAN) with peripheral neuropathy are reported. Diagnosis was based on pathological criteria of necrotizing vasculitis in 20 patients (group I) and on clinical, biological and sometimes arteriographic data in 7 (group II). The 27 patients (18 women and 9 men), aged between 30 and 78 years (mean: 53.4 years), presented either a mono- or multineuropathy (8 cases), a polyneuropathy (17 cases), or sensory symptoms (2 cases). A neuropathy was the initial feature of the PAN in 15 cases. The CSF, examined in 16 cases, was always normal, and results of electrophysiological investigations constantly suggested the presence of an axonal neuropathy with normal motor nerve conduction rates. Other signs of PAN included fever (81 p. 100 cases) and loss of weight (74 p. 100). The ESR was increased in 70 p. 100 cases and hypereosinophilia was present in 33 p. 100. Quantitative pathology in 13 cases from group I revealed typical findings, and associated microvasculitis in 4 cases. The outcome was fatal in 13 cases while 12 recovered and have been followed up for from 1 to 14 years.

Adrenal Cortex Hormones↗

[Pedicled craniopharyngioma of the 3d ventricle].

A 62 year-old man suffered of headache and progressive walking difficulties for 4 years. Radiological examinations showed a calcified intraventricular tumor attached to the floor of the 3rd ventricle. Death, caused by septicemia, occurred before neurosurgery. On sagittal braincut the tumor appeared pediculated and was attached to the anterior part of the floor of the 3rd ventricle. The microscopic features were those of typical craniopharyngioma. The pedicle and the floor of the 3rd ventricule were devoid of tumoral cells. Numerous large vessels which originated in the basal leptomeninges were present in the ventricular floor and the pedicle and then branched out into the tumor. Pathologically proven purely intraventricular craniopharyngiomas have been seldom reported. To our knowledge an autopsy case of pediculated intraventricular craniopharyngioma has been previously described only once, without particular attention to the pedicle. The integrity of the floor of the 3rd ventricle constitutes the only feature that may differentiate with certainty an intraventricular extension of a suprasellar craniopharyngioma from a pure intraventricular form of this tumor.

Cerebral Ventricle Neoplasms↗

[Nerve biopsy. Advantages and limitations of modern examination techniques (author's transl)].

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.

Amyloidosis↗

Peroneal muscular atrophy. Part 1. Clinical and electrophysiological study.

144 patients with the clinical syndrome of peroneal muscular atrophy or Charcot-Marie-Tooth disease were studied. Thirteen were recognized as the spinal form of the disease since all had normal motor nerve conduction velocity and sensory nerve action potential. The remaining patients could be classified according to their values of motor conduction velocity for the median nerve. Two main groups were therefore identified: 55 patients whose nerve conduction was below 30 m/s belonged to group I, which corresponds to the previously reported hypertrophic form or hereditary motor sensory neuropathy (HMSN) type I. Sixty-four patients whose nerve conduction was above 40 m/s belonged to group II which corresponds in the majority of cases to the neuronal form or HMSN type II. Twelve patients could not be classified since the motor nerve conduction velocity for median nerve ranged between 30 and 40 m/s. These could belong to either of the two main groups or might form an intermediate group, the existence of which is discussed. Clinical genetic and electrophysiological features of the two main groups are discussed and compared.

Adult↗

Peroneal muscular atrophy. Part 2. Nerve biopsy studies.

The present study used the nerve pathology, studied by quantitative light microscopy, as the sole basis for classification of peroneal muscular atrophy (PMA). The findings in biopsies of superficial peroneal nerves of 20 patients were compared with normal values obtained from 8 controls. Three homogeneous groups comprising 17 out of 20 patients were clearly identified. The hypertrophic type (7 cases) was characterized by (1) many multilamellated onion bulb formations; (2) extensive loss of MF with both involvement of the largest and smallest fibers; (3) lack of significant cluster formation. The neuronal sensori-motor type (5 cases) was characterized by (1) absence of any OB; (2) elective loss of large MF; (3) abundance of clusters with significant increase of the small MF population. The neuronal motor type (5 cases) showed a virtually normal sensory nerve except for fairly numerous clusters in some cases. Comparing this classification based on histomorphometric grounds, with the electrophysiological data it appears that 14 patients out of 17 would be correctly classified as hypertrophic or neuronal with respect to the motor nerve conduction velocity of the median nerve alone. Three cases were not classified in the previous groups since they differed notably in one or more parameters from the typical cases. A possible intermediate group is discussed.

Adult↗

The slim disease in African patients with AIDS.

Ten unselected African patients infected with human immunodeficiency virus (HIV) and with slim disease were evaluated using physical examination, anthropometric measurements, Karnovsky performance score, and muscle biopsy. All had marked weight loss (36.8 +/- 10.8%) with extreme fatigue, marked diffuse wasting with significantly decreased circumferences of arms, thighs and calves (P < or = 0.002), and a low Karnovsky performance score (range 30-70). Mild to moderate motor deficit (in 9/10 patients) contrasted with the major amyotrophy. Chronic diarrhoea (in 7/10) and/or prolonged fever (in 7/10) were always associated with the amyotrophy. Atrophy of muscle fibers was the main finding of muscle biopsy. Only 5 patients met the CDC criteria for the 'HIV wasting syndrome'. We conclude that slim disease, which is highly suggestive of the acquired immune deficiency syndrome (AIDS) in Africa, is a condition associated with chronic diarrhoea and/or prolonged fever, that encompasses the 'HIV wasting syndrome' sensu stricto and probably other debilitating diseases associated with AIDS, such as tuberculosis.

Acquired Immunodeficiency Syndrome↗

Pathology of the central nervous system in 40 cases of acquired immune deficiency syndrome (AIDS).

The central nervous system was examined in 40 AIDS patients who died between August 1982 and 1987. The cases included two children born to intravenous drug abusers and 38 male adults. The brains of eight patients who had no clinical or radiological evidence of central nervous system involvement showed non-specific changes which included microglial nodules, perivascular mononuclear cuffs, mineralization of blood vessels and granular ependymitis. In 32 brains from patients with neurological symptoms, toxoplasmosis was the most frequent finding (19 cases) manifested by multifocal, necrotic lesions or a diffuse pseudo-encephalitic process. Other opportunistic infections included cytomegalovirus (eight cases), progressive multifocal leucoencephalopathy (two cases), cryptococcosis (one case), aspergillosis (one case), multiple bacterial microabscesses (one case) and Mycobacterium avium intracellulare (one case). Two patients had cerebral lymphoma. Subacute encephalitis with white matter lesions and multinucleated giant cells characteristic of HIV infection was present in 15 cases. Various combinations of all these infections were encountered in the same brain, sometimes in the same area and, occasionally, in the same cell.

Acquired Immunodeficiency Syndrome↗

Expanding cerebellar lacunes due to dilatation of the perivascular space associated with Binswanger's subcortical arteriosclerotic encephalopathy.

An 80-year-old hypertensive woman developed right hemiplegia and died 24 hours after admission. Neuropathologic examination revealed multiple cerebral infarcts of various ages and diffuse subcortical arteriosclerotic encephalopathy. Clusters of asymptomatic "expanding" lacunes, due to dilatation of the perivascular spaces, were found in both dentate nuclei. These cavities, which presented as space-occupying lesions, were surrounded by a single layer of flattened cells and contained 1 or more sections of normal-looking arterioles. Such a topographic grouping of lacunes in the dentate nucleus has not been described previously. The mechanism of widening of the perivascular compartment remains unclear; its occurrence in a hypertensive patient and its association with typical Binswanger's subcortical arteriosclerotic encephalopathy and severe atherosclerosis with multiple infarcts suggest a common pathophysiologic mechanism possibly including an alteration of the blood-brain barrier.

Aged↗