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Cavernous angioma: a review of 126 collected and 12 new clinical cases.

The histopathological, clinical, and radiological features of the intracranial cavernous angioma are reviewed, based on an analysis of 138 symptomatic, histologically verified cases. Twelve of the cases are from our own series and 126 were collected from appropriately documented reports in the modern literature. The analysis indicated that, at the time of diagnosis, one-third of the patients (49 cases) were being evaluated for seizures, one-third (40 cases) for clinical evidence of hemorrhage, and one-third (49 cases) for mass lesions. Unlike the other two groups, the group presenting with clinical evidence of hemorrhage was distinguished by a dominant age at the time of diagnosis (41% were diagnosed during the 4th decade of life), by a high incidence of prior neurological evaluation (43%), by a higher rate of diagnosis at autopsy (28%) than at operation, and by the absence of microscopic calcification within the lesion.

Adolescent↗

A history of Todd and his paralysis.

OBJECTIVE: To describe the history of Robert Bentley Todd (1809-1860) and certain of his contributions to medicine, including his original and subsequent descriptions of "epileptic hemiplegia," which came to be called "Todd's paralysis." METHODS: The author conducted a comprehensive review of English-language literature, modern and historical, related to "Todd's paralysis" and examined Todd's original case histories and commentary by Todd, his contemporaries, and his successors. RESULTS: Todd held that some patients "who recover from a severe fit, or from frequently repeated fits of epilepsy, are often found to labor under hemiplegia, or other modifications of palsy." He believed that this resulted from "undue exaltation. [resulting in] a state of depression or exhaustion." Interestingly, Todd was the first to present an electrical theory of epilepsy, supported by his own animal experimentation, well before his better-known successor John Hughlings Jackson (1835-1911) (famous for his investigations of partial epilepsies and the eponymous "Jacksonian march"). CONCLUSION: Many neurologists and investigators followed Todd in acknowledging transient postictal paralysis as a distinct clinical entity. Yet whether the pathophysiology of "Todd's paralysis" is related to "neuronal exhaustion" or excessive inhibition is still controversial.

History, 19th Century↗

T/NK cell non-Hodgkin's lymphoma of the sinonasal tract.

Non-Hodgkin's lymphoma of the sinonasal tract is now recognized as an important cause of destructive midfacial lesions formally designated as idiopathic inflammatory processes, and commonly treated with local radiotherapy in a bid to halt the destructive process. However, left untreated, the natural history of this disease remains largely unknown. We report a case which demonstrates the slow and apparently indolent natural course that these lymphomas, if left untreated, may display, before finally evolving into overwhelming and fatal disease. We also take the opportunity to present a brief synopsis of the evolution of our understanding of this condition and to review the modern literature on it.

Disease Progression↗

"Contarini's condition:" bilateral pleural effusions with markedly different characteristics.

A leukemic patient presented with bilateral pleural effusions. The effusions were markedly different in their characteristics; left-sided staphylococcal empyema and right-sided lymphocytic effusion. To our knowledge, there are no cases of this type reported in the modern literature. The importance of bilateral diagnostic thoracocentesis in patients on immunosuppressant therapy is emphasized.

Empyema↗

Sympathetic sprouting in dorsal root ganglia (DRG): a recent histological finding?

During the nineties it was described, as an original finding, the existence of afferent amyelinic nerve endings in animal dorsal root ganglia (DRG) caused by diverse experimental lesions. These works do not take into account the historical studies carried out by Ehrlich (1886), Ramón y Cajal (1890) and Dogiel (1885) among others. Ramón y Cajal (1899) confirmed the existence of these nerve endings naming them after their discoverer as "Dogiel's arborisations". Ramón y Cajal claims that these endings originate from fibres of sympathetic nature, something supported by later authors devoted to this topic. In any case, the same authors remarked already a possible relationship with pathological phenomena, nonetheless always referring to the frequent occasions in which the same images appeared in healthy animals. In this work we review the bibliography about the classically named "Terminal Dogiel's nests" which in modern literature have been referred to as sprouting of sympathetic axons in dorsal root ganglia likely related with sympathetically maintained pain. Furthermore, we present the finding, not described up to date, of multiple afferent amyelinic nervous endings related with the "Terminal Dogiel's nests" observed in different DRG from young adult healthy rabbits.

Animals↗

[Clinical pathology].

This work describes the basic elements of pathology used in clinical practice. Pathology plays an important role in clinical and scientific work, but only a few areas of pathology will be covered. Although the contribution of oncological and surgical pathology to therapy is the most well known, the cases chosen here will involve infectious pathology, diseases of the kidney and the liver, autoimmune diseases, as well as organ transplantation. Especially important is the description of methods that enable more accurate morphological diagnoses, such as histochemistry, immunohistochemistry, immunofluorescence, and electronic microscopy. Previous experience and joint work with clinical doctors have enabled the definition of significant morphological elements as well as of essential methods of pathohistological diagnosis. Besides, as is often the case, although disease symptoms are difficult to discern and biochemical results do not show significant changes compared to normal values, the results of biopsy come as a surprise to clinical doctors. For example, in virus hepatitis B involving so-called asymptomatic HBsAg carriers, we discovered every morphological form of hepatitis, from minimal lesions to chronic, persistent, and active hepatitis. With hepatitis C, certain morphological lesions point to the etiopathogenesis of this disease and thus help to confirm the diagnosis and to instigate therapy on time. Another significant experience involves kidney biopsies in cases when clinical findings are asymptomatic. Often, in such cases, morphological findings point to glomerulonephritis and glomerulopathy at different stages. Timely and subtle morphological diagnostics offer a more precise explanation for the pathological injury of tissues than other diagnostic methods. In this way, by adopting new methods, the work of pathologists is included more and more in everyday clinical practice. The inclusion of pathologists in a transplantation team makes sure a proper selection of the organ for transplantation is carried out and ensures a reliable evaluation of the condition of the transplanted organ, enabling appropriate therapy. Autoimmune, hereditary diseases are almost impossible to recognise unless a biopsy is performed as in the examples given. In this work, the 30-year-long results of the cooperation between clinical doctors and pathologists are presented and compared with similar results from modern literature, together with numerous examples that represent significant experiences and achievements of our medicine.

Humans↗

Kashin-Beck disease. An analysis of research and public health activities based on a bibliography 1849-1992.

The main feature of Kashin-Beck disease (KBD) is short stature caused by multiple focal necroses in the growth plate of the tubular bones. This leads to a secondary, sometimes severe osteoarthrosis. The disease was first described in Russia from the Bajkal area by Kashin 1848 and later, 1906, by Eugene Beck. Modern literature is mainly from China. The main theory originally proposed by Russian investigators was that KBD was caused by a toxic effect of mycotoxin. The focus on the disease gradually shifted to China where the causal theory has been based on the effects of selenium deficiency and its interaction with mycotoxins. These main hypotheses may be unified in one theory. KBD is appreciated as a public health problem estimated to affect some 1-3 million people across China. National and provincial registers on KBD exist for selected affected areas. Current incidence is probably declining. Preventive efforts have been undertaken but are usually not controlled for effectiveness nor by putative causal mechanisms. More research into the causal mechanisms is required. A combination of difficulties to obtain literature, including primary material together, and cultural and language barriers have been a main obstacle to more active participation by the international research community. This bibliography is an attempt to help improve this situation. The bibliography covers the period 1849-1992. The analysis of this bibliography shows a broad and intensive, though sometimes insufficiently coordinated research. Many authorities and research bodies are involved. The possibility to develop coordinated projects within a common framework, and thus to use research results to prevent KBD, has been a major source of inspiration in making this review. The review covers 499 publications directly related to KBD and 1415 references to these publications. KBD publications have been retrieved from conventional international journals, usually in English, publications in Russian, as well as unpublished reports in Chinese provided at site visits. The papers are classified by key words. The references in these publications have also been similarly classified and are in part analysed. Based on publications identified from several sources, direction of research and public health efforts from 1848 to 1992 are described. Finally, mainly based on questions raised by and recommendations from a WHO workgroup meeting in 1992, some major components of research activities and public health program are proposed.

Animals↗

The wrath of grapes versus the self-medication hypothesis.

Dr. Khantzian's SMH was initially developed to enhance an etiologic understanding of substance use disorders and to promote appropriate and empathic therapeutic responses through careful listening to the human suffering of addicted patients. In the present review he has combined an intensive look at patients with a full exploration of the modern literature on comorbidity and a discussion of the ways in which this more recent work may support or contradict the SMH. Additional longitudinal research is needed to confirm and delineate the SMH. There is a danger that clinicians could use the SMH in overly reductionistic ways. They must be alert to potential rationalization and retrospective distortion while listening to their patients' accounts of the causes of their substance abuse. In the cart/horse problem, it can be clinically useful to tease apart the temporal sequence of onset of each disorder, the ways in which the two disorders interact, and the ways in which each may amplify the suffering caused by the other. In my view, it is still the wrath of grapes that leads to the greater part of suffering in substance use disorders.

Humans↗

Reptile envenomations.

Venomous reptiles are distributed in select habitats in temperate and tropical areas of the world with few geographical exceptions, and have adapted to not only terrestial existence, but to arboreal and aquatic environments as well. Venomous snakes are found in the families Colubridae (fixed and rear fanged snakes), Elapidae (fixed and front fang snakes), Hydrophiidae (sea snakes), Viperidae (Old World vipers) and Crotalidae (pit vipers). Venomous lizards are found in the United States and Mexico, and comprise the family Helodermatidae. Venom delivery systems and venom components show diversity, and greater appreciation of interspecies clinical effect is apparent in modern literature. First aid care for the bitten individual remains controversial, but most authorities now tend to minimize field procedures, especially those endeavors which may potentially damage tissue. The weight of evidence in the area of definitive therapy lies with the use of antivenin, although proponents of primary surgical intervention in crotalid envenomations have followings, particularly in the United States. Recent developments in "purification" of existing antivenins are promising, and attention to species-specific antivenin production, especially to the venoms of the crotalid species of the New World, is encouraged. Due to a growing international traffic in venomous animals for the purposes of research and supply of zoos and private reptile collections, knowledge of resources for assistance in bites of non-indigenous reptiles is a growing consideration.

Animals↗

Primitive neuroectodermal tumor of the median nerve. Case report with cytogenetic analysis.

The authors describe a malignant peripheral primitive neuroectodermal tumor (PNET) that originated in the median nerve in an elderly adult. After the diagnosis was made by biopsy, the patient underwent radical local resection with interpositional vein grafting of the brachial artery. The tumor had the typical appearance of a primitive neural tumor with small, round cells forming rosettes. It stained positively for both the Ewing's sarcoma/peripheral PNET antigen (HBA-71) and neuron-specific enolase, confirming its neural origin. Ultrastructural examination revealed dense core granules and suggested neural differentiation of the neoplasm. Cytogenetic analysis suggested a chromosome (11;22) translocation typical of peripheral PNET. Early reports consisted of tumors arising solely in peripheral nerves, but recent series have focused mainly on tumors arising in the soft tissues other than nerves. There are no other cases of true PNET of peripheral nerve in the modern literature that have been fully characterized by immunohistochemical, ultrastructural, and cytogenetic criteria. Although peripheral PNETs occur more commonly in children, this unusual neoplasm should be considered in the differential diagnosis of peripheral nerve neoplasms in adults. Early diagnosis is desirable because of its aggressive nature and poor outcome.

Aged↗

Infective lumbar discitis in a sickler--an occult 'typhoid' spine?

Pyogenic infection of the intervertebral disc (discitis) is a rare infection and the diagnosis often depends on a high index of suspicion. The cases of infective discitis described in the modern literature are similar to, if not identical with what was described as 'typhoid spine'. Salmonella infection of the musculoskeletal system on the other hand is more common in patients with sickle cell anaemia. This case report highlights the bizarre presentation of infective lumbar discitis in a sickler (HbSS) and calls attention to the need for a thorough evaluation of low back pain in these patients.

Adult↗

[Clinical and hematogenic considerations on 2 cases of cerebral venous thrombosis occuring during the use of oral estro-progestational preparations].

The authors illustrate the clinical peculiarities and the laboratory data of thrombotic accidents as complications of oral contraceptives and other estrogen-containing drugs, concerning modern literature. Moreover, they describe two cases of cerebral venous thrombosis verified in Neuropathology Department of Maria Vittoria Hospital-Torino. The problem of prevention of these accidents is debated in the second part of the article.

Adult↗

[Evaluation of psychological disorders in patients with cancer of the oral cavity].

BACKGROUND AND AIMS: This study aimed to evaluate the possible onset of forms of anxiety, major and minor depression and behaviour towards the disease shown by patients with oral cavity neoplasms. METHOD: An explorative comparative study was performed in 28 patients referred to the department of Stomatological Surgery at the Odontostomatology Clinic of Turin University. Ten patients were examined before surgery and 18 after. The tests were performed using Hamilton's scale, STAI X1 and X2 and IBQ. These tests were carried out either during hospitalisation or during outpatient check-ups by an interviewer who was not familiar with the diagnosis or the patient's personal history. RESULTS: In the group of subjects evaluated before surgery, there was a scant presence of psychological correlations to disease. This shows that negation is widely used. Almost all subjects revealed the presence of anxiety symptoms and 60% of subjects were affected by minor depression. The level of negation was even higher in the postoperative group. This shows that the characteristics of the defensive structure did not change. CONCLUSIONS: Patients showed a psychological structure, even some time after surgery, which was similar to that in the first group of subjects, namely those facing an active phase pathology. The above findings underline the need for greater attention to be paid to the quality of life of these subjects who, owing to improved treatment, are becoming increasingly numerous. The scores obtained show that the majority of these patients encounter major difficulties at a psychological level, even if they manage to overcome the disease in physical terms. In conclusion, this study highlights the need to offer these subjects valid psychological support during diagnostic and therapeutic procedures. It also underlines the need for longitudinal studies in larger and more homogeneous groups of patients suffering from this disease since they appear to be somewhat neglected by modern literature.

Anxiety↗

Intracerebral malignant fibrous histiocytoma in a 5-year-old girl.

A case of primary intracerebral malignant fibrous histiocytoma (MFH) in 5-year-old girl is presented, the eighteenth case so described in modern literature. A lobulated, heterogenous mass lesion with a haemorragic component was present in our case' s MRI. A review of the literature on MFH of the pediatric age group was done to establish guidelines for standard treatment modalities in primary intracerebral MFH.

Brain Neoplasms↗

Parietal lobe epilepsy.

When symptoms such as lateralized paresthesias or pain occur prominently and early in partial seizures, parietal lobe seizure origin should be suspected. Most patients with parietal lobe seizures, however, have no symptoms or signs suggesting the parietal lobe. In the absence of detectable epileptogenic lesions, these patients without clinical seizure characteristics suggesting parietal lobe origin can present with misleading findings, resulting in erroneous localization, which can, in turn, lead to ineffective surgical intervention (23,25). Although ictal SPECT might provide vital evidence of parietal lobe seizure origin (25), as noted previously, this technology also can produce misleading data in some patients (52). Even when parietal lobe seizure origin is suspected, in the absence of a structural lesion, documenting this with invasive monitoring can be difficult. The parietal lobes, like the frontal lobes, are large, diffuse structures, and the potential for sampling error is high (19). Spread patterns are unpredictable and can result in false localization (23). Even with extensive and repetitive invasive studies, localization can prove elusive (53). No well-documented series of patients with non-lesional parietal lobe epilepsy in the modern literature who have been cured by surgery have been reported as a result of a combination of the rarity of the condition, the lack of correct recognition, as well as the difficulty of localization. Patients with medically intractable parietal lobe seizures, however, can experience excellent surgical results (23,24). Postoperative parietal lobe symptoms and signs, even when extreme, are usually not enduring (patient 3). One of our patients, however, did develop a chronic pain syndrome (48).

Epilepsy↗

[Corticosteroid receptors in the brain as signal systems of stress and adaptation].

The modern literature date and own authors investigation allow to consider brain corticosteroid receptors as the importance system of stress and adaptation. The main attention is paid for functional meaning and distribution of corticosteroid receptors in brain regions. The role of brain corticosteroid receptors in transduction of hormonal signal and its modification different regulatory factors are discussed.

Adaptation, Physiological↗

[Cells-chandeliers and axo-axonal inhibition in the neocortex, hippocampus, and the dentate gyrus].

An exceptional category of cortical non pyramidal interneurons--chandelier cells or axo-axonal cells or axo-axonal cells exert specific inhibition at the level of initial segment of pyramid axons. There are evidences that chandelier cells are directly involved in the development of Alzheimer disease, a degenerative brain pathology. Morphological features and neurochemical specialization, history of opening of chandelier cells was described as well as their links in neocortex, hippocampus and dentate fascia of man and animals. Basing on results of the authors study and the findings available in literature modern conceptions on histophysiology of axo-axonal inhibition were critically analysed. It was emphasized that the function of chandelier cells is highly specified--they prevent occasional cell connections and serve as a factor of nervous system regulation.

Animals↗

[Our experience with laminectomy in the treatment of spinal cord disease caused by cervical arthrosis: apropos of 42 cases].

Cervical spondylotic arthRosis may lead to a myelopathy and the question which can rise is: posterior surgical decompression is adequate regarding the others surgical technics discussed? In this paper the authors describe the postoperative results of 42 patients who have been operated on by laminectomy from 1971 to 1995 at Fann Hospital in Dakar. The median age was 42 years and the median delay between the onset of neurological disorders and operation was 5.9 months. All patients showed symptoms of spinal cord and root disturbances. Neuro-imaging studies with plan X-rays and myelography reveal arthrosis and the impact of this arthrosis on nervous system. With a follow up of 6 months we found a mortality rate of 4.7% (two cases of death), 66.8% of good results and 28.5% of fail. Eighteen months post surgery results were respectively 52.3% and 45.7% of good and poor results. The complications of the laminectomy were peri operative hemorrhage (19%); sepsis (21.4%); spine cord traumatic injuries (9.5%) spondylolisthesis (4.7%). Laminectomy has been found to improve "spine syndrome"; paresthesia; fasciculations, and sensory deficits. Also three level laminectomy and surgery which can take place before six months are good prognosis factors. The authors stressed on laminectomy because of our low medical care situations particularly that no other surgical procedures through the modern literature leads to better results.

Adult↗