Vasculitis and collagen vascular diseases

Author(s):  
Neil Scolding

That part of the clinical interface between neurology and general medicine occupied by inflammatory and immunological diseases is neither small nor medically trivial. Neurologists readily accept the challenges of ‘primary’ immune diseases of the nervous system: these tend to be focussed on one particular target such as oligodendrocytes or the neuro-muscular junction present in predictable ways, and are amenable as a rule to rational, methodological diagnosis, and occasionally even treatment. This is proper neurology.‘Secondary’ neurological involvement in diseases mainly considered systemic inflammatory conditions—for example, SLE, sarcoidosis, vasculitis, and Behçet’s—is a rather different matter. It may be difficult enough to secure such a diagnosis even when systemic disease has previously been diagnosed and new neurological features need to be differentiated from iatrogenic disease, particularly drug side effects or the consequences of immune suppression. But all the diseases mentioned may present with and confine themselves wholly to the nervous system; they may mimic one another, and pursue erratic and unpredictable clinical courses. In central nervous system disease, diagnosis by tissue biopsy is potentially hazardous and unattractive. Few neurologists enjoy excesses of confidence or expertise when faced with such clinical problems: the cautious diagnostician is perplexed, and the evidence-based neuroprescriber confounded. Unsurprisingly, great variations in approaches to diagnosis and management are seen (Scolding et al. 2002b).But rheumatologically inclined general, renal or respiratory physicians, comfortable when managing inflammation affecting their system or indeed other parts of the body designed to support the nervous system, are generally also ill at ease when faced with neurological features whose differential diagnosis may be large, particularly given the near universal diagnostic non-specificity of either imaging or CSF analysis.Here then is the subject material for this chapter: the diagnosis and management of central nervous system involvement in inflammatory and immunological systemic diseases (Scolding 1999a). In not one of these neurological conditions has a single controlled therapeutic trial been reported, and much that is published on these conditions is misleading or inaccurate. And yet the frequency with which the diagnosis is only confirmed or even first emerges at autopsy bears stark witness to both the severity and evasiveness of these disorders.

2020 ◽  
Vol 13 (9) ◽  
pp. e235412
Author(s):  
Jesse Mooneyham ◽  
Cesar Gentille ◽  
Andrea Barbieri ◽  
Shilpan Shah

A 33-year-old woman presented to the emergency room with severe headaches. A CT scan of the head revealed two brain lesions with associated vasogenic oedema. Diagnostic resection of one of the lesions followed by pathological analysis revealed grade III lymphomatoid granulomatosis (LYG). Staging investigations elsewhere in the body were negative, isolating this case of LYG to the central nervous system, an atypical presentation. After the resection, she was treated with single-agent rituximab 375 mg/m2. The follow-up MRI demonstrated the resolution of brain lesions and no progression of the disease.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Shanila Ahmed ◽  
Babar Irfan ◽  
Muhammad Raza ◽  
Ghulam Haider

Abstract Background Hodgkin lymphoma is a systemic disease that commonly involves the cervical, supraclavicular, and mediastinal lymph nodes. The involvement of central nervous system in Hodgkin lymphoma is extremely rare, and diagnosis is usually established using distinct morphological and immunohistochemical staining on the tissue biopsied. Extranodal presentation of HL is a rare occurrence. It has been evident that prognosis is encouraging in patients with disease that is limited to just central nervous system initially or as relapse, compared with involvement of multiple sites of relapse. Case presentation We herein report a case of a 35-year-old South-East Asian male with relapsed Hodgkin lymphoma. The patient developed a parotid gland lesion, cervical lymphadenopathy with significant weight loss, and intermittent night sweats. Along with spread to the central nervous system, there was a high suspicion of tuberculosis. Upon biopsy of his cervical lymph node, the patient was confirmed to have Hodgkin lymphoma. Immediate treatment began with six cycles of chemotherapy consisting of adriamycin, bleomycin, vinblastine, and dacarbazine. The patient received three cycles of chemotherapy consisting of ifosfamide, carboplatin, and etoposide but then was lost to follow-up. Five years later, the patient suffered a road traffic accident. Upon work-up, a right parietal space-occupying lesion with moderate cerebral edema and midline shift was found on computed tomography of the brain. The patient underwent resection of the space-occupying lesion of brain, with features consistent with classical Hodgkin lymphoma on histopathology examination. It is crucial for such lesions to be investigated meticulously to rule out any secondary disease process. Conclusion Relapsed Hodgkin lymphoma with central nervous system involvement is relatively rare with just over two dozen cases reported to date and is observed infrequently in developing nations. Therefore, space-occupying lesion should always be investigated, and biopsy of such lesions is gold standard to establish diagnosis. With timely appropriate therapy, complete remission can be achieved. However, large-scale studies would be prudent to explore the presentation, survival, and treatment options for patients with Hodgkin lymphoma involving the central nervous system.


2018 ◽  
Vol 76 (4) ◽  
pp. 435-438
Author(s):  
Sofia Lopes ◽  
Júlia Vide ◽  
Sofia Magina ◽  
Ana Paula Cunha ◽  
Filomena Azevedo

Pyoderma gangrenosum is a chronic inflammatory disease characterized by the development of a painful deep ulcer with undermined borders. Head and neck are rarely affected regions of the body and also usually associated with a worse prognosis. Corticosteroids are the mainstay of treatment although available options are not specific nor completely effective in pyoderma gangrenosum. We report the case of a 46-year-old patient with an aggressive orbital pyoderma gangrenosum with progressive extension to the central nervous system and insufficient response to treatment, ultimately leading to patient’s death.


Blood ◽  
1965 ◽  
Vol 25 (1) ◽  
pp. 1-12 ◽  
Author(s):  
CAROL B. HYMAN ◽  
JAMES M. BOGLE ◽  
CHARLES A. BRUBAKER ◽  
KENNETH WILLIAMS ◽  
DENMAN HAMMOND

Abstract Observations on the course of 59 children who experienced 109 distinct episodes of CNS involvement by leukemia showed that: 1. This complication may be associated with all types of acute and subacute leukemia. 2. There is no single or combination of diagnostic criteria. Manifestations of increased CSF pressure, such as vomiting, headache, and papilledema are the most frequent clinical findings. However, it should be emphasized that CNS involvement may be associated with normal CSF findings. 3. CNS involvement may be present at the onset of leukemia or can occur at any time during the course. 4. Approximately 26 per cent of children with leukemia develop CNS involvement. 5. CNS involvement may occur when the disease is under apparently good therapeutic control as well as during relapse. 6. There is no relationship between the agents which had been previously used to treat the systemic disease and the later development of CNS involvement. However, the onset of CNS symptoms was less frequent when the systemic disease was under treatment with steroids. 7. The development of CNS involvement does not appear to shorten the survival time of patients with leukemia when treatment for CNS involvement is given.


2015 ◽  
Vol 9 (1) ◽  
pp. 21-23 ◽  
Author(s):  
Ioannis Zalonis ◽  
Foteini Christidi ◽  
Constantin Potagas ◽  
Michalis Rentzos ◽  
Ioannis Evdokimidis ◽  
...  

Whipple’s disease (WD) is a rare systemic disease caused by the gram-positive bacillus Tropheryma Whipplei and mostly characterized by arthralgias, chronic diarrhea, weight loss, fever and abdominal pain. Central Nervous System involvement is not uncommon and it may precede other disease manifestations, appear after treatment and improvement of gastrointestinal signs or rarely be the only WD symptom. We report a case in a middle-aged male with unexplained neurological signs and symptoms which were presented as relapse of previously undiagnosed WD with atypical symptoms at onset. After diagnosis confirmation, the patient was appropriately treated which resulted in improvement of major symptoms.


Author(s):  
F. L. Azizova ◽  
U. A. Boltaboev

The features of production factors established at the main workplaces of shoe production are considered. The materials on the results of the study of the functional state of the central nervous system of women workers of shoe production in the dynamics of the working day are presented. The level of functional state of the central nervous system was determined by the speed of visual and auditory-motor reactions, installed using the universal device chronoreflexometer. It was revealed that in the body of workers of shoe production there is an early development of inhibitory processes in the central nervous system, which is expressed in an increase in the number of errors when performing tasks on proofreading tables. It was found that the most pronounced shift s in auditory-motor responses were observed in professional groups, where higher levels of noise were registered in the workplace. The correlation analysis showed a close direct relationship between the growth of mistakes made in the market and the decrease in production. An increase in the time spent on the task indicates the occurrence and growth of production fatigue.Funding. The study had no funding.Conflict of interests. The authors declare no conflict of interests.


Author(s):  
Prithiv K R Kumar

Stem cells have the capacity to differentiate into any type of cell or organ. Stems cell originate from any part of the body, including the brain. Brain cells or rather neural stem cells have the capacitive advantage of differentiating into the central nervous system leading to the formation of neurons and glial cells. Neural stem cells should have a source by editing DNA, or by mixings chemical enzymes of iPSCs. By this method, a limitless number of neuron stem cells can be obtained. Increase in supply of NSCs help in repairing glial cells which in-turn heal the central nervous system. Generally, brain injuries cause motor and sensory deficits leading to stroke. With all trials from novel therapeutic methods to enhanced rehabilitation time, the economy and quality of life is suppressed. Only PSCs have proven effective for grafting cells into NSCs. Neurons derived from stem cells is the only challenge that limits in-vitro usage in the near future.


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