scholarly journals Lhermitte duclos disease: what to expect during surgery?

2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Sanjeev Kumar ◽  
Debabrata Sahana ◽  
Amit Jain ◽  
Lavlesh Rathore ◽  
Manish Tawari ◽  
...  

AbstractLhermitte and Duclos first described the dysplastic gangliocytoma of the cerebellum in 1920. In the last 100 years, its clinical presentation, radiological features, pathological characteristics, and association with Cowden syndrome have been well described. However, documentation of surgical experiences is lagging. We here describe intraoperative experience during the removal of the tumor, which could help the operating surgeon plan and mental makeup.

Neurosurgery ◽  
2004 ◽  
Vol 55 (6) ◽  
pp. E1430-E1434 ◽  
Author(s):  
Carlo Santaguida ◽  
Abdulrahman J. Sabbagh ◽  
Marie-Christine Guiot ◽  
Rolando F. Del Maestro

Abstract OBJECTIVE AND IMPORTANCE: Intramedullary melanotic schwannomas are very rare lesions; only four cases have been reported previously. We describe a patient with an intramedullary melanotic schwannoma that had a more aggressive course than those reported in the literature, and we review the theories regarding the cause of these lesions. CLINICAL PRESENTATION: A 35-year-old man presented with neck stiffness and paraesthesia extending down his right arm upon neck extension. A magnetic resonance imaging scan revealed an intramedullary lesion extending from C4 to C5. INTERVENTION: Gross total resection of the mass was performed, and pathological characteristics were consistent with a melanotic schwannoma. Two years after resection the tumor recurred, and the patient was treated with radiation therapy. The tumor progressed 2 years after radiotherapy, and at repeat resection, multiple pigmented foci were present on the surface of the spinal cord and dura consistent with metastatic seeding. CONCLUSION: In a patient with intramedullary melanotic schwannoma with an unusually aggressive course, careful follow-up may be essential.


Author(s):  
Nidhish Kumar ◽  
Sharvani Singh ◽  
Bipin Kumar

Lhermitte and Duclos first described Dysplastic Gangliocytoma in 1920. Lhermitte Duclos Disease (LDD) is an extremely rare disorder of uncertain prognosis and pathogenesis. LDD is recognised as a part of Cowden Disease, which being an autosomal-dominant phacomatosis and cancer syndrome. Till date only about 225 cases of LDD have currently been reported in medical literature. It is most commonly seen in young adults with a peak incidence in third or fourth decade with signs and symptoms of cerebellar dysfunction or increased intracranial pressure leading to obstructive hydrocephalus. A 36-year-old female came to Neuromedicine Outpatient Department (OPD) in a Tertiary Care Superspecialty Hospital with chief complaints of headache and vomiting, difficulty in swallowing liquid food since two months with no significant family history. The patient’s general condition was not good with significant weakness on presentation. The patient was conscious, oriented with pulse rate of 78/bpm, blood pressure of 118/80 mm of Hg with bilateral clear chest with normal S1 S2 sound. The Glasgow Coma Scale (GCS) was found to be Eye response-4, Verbal response-5, Motor response-6. Magnetic Resonance Imaging (MRI) of patient showed large heterogeneous non-enhancing lesion involving left cerebellar hemisphere, vermis and cerebellar peduncle with widened cerebellar folia with a “Tigroid appearance”. A diagnosis of Cerebellar Gangliocytoma was made and was treated successfully with surgery and diagnostically proven with biopsy and immunohistochemistry. The background history of Cowden syndrome was not present in index case.


2020 ◽  
Vol 40 (1) ◽  
pp. 38-40
Author(s):  
Petra Bago Rožanković ◽  
Maristela Stojić ◽  
Jasna Badžak

Acute disseminated encephalomyelitis (ADEM) is an inflammatory, demyelinating disease of the central nervous system that occurs as an immunological response to a viral or bacterial infection or an immunization. We describe a patient with clinical presentation and radiological features of ADEM that appeared after a spider bite. Corticosteroid therapy did not produce satisfied treatment response. The patient recovered after five days of immunoglobulin therapy. The cross-reactivity between spider toxin and myelin could explain pathophysiological mechanism of demyelination. ADEM should be considered as a possible complication of a spider bite.


2018 ◽  
Vol 37 (02) ◽  
pp. 119-122
Author(s):  
Miguel Maldonado-Morán ◽  
Jeisson Ospina ◽  
Juan Vega ◽  
Claudia Restrepo ◽  
Daniela Rico ◽  
...  

AbstractPituicytoma is a rare tumor that arises from the glial cells of the neurohypophysis. For a long time, it was believed that pituicytomas only appeared in adults. Currently, at least three cases of this entity occurring in children have been reported in the literature. The aim of the present report is to describe the case of a 5-year-old girl who presented to the emergency department with visual disturbances, and the diagnosis was a recurrent pituicytoma. Therefore, the clinical presentation, the radiological features of the tumor, and the corresponding surgical management are described. Additionally, a brief review of the management of this unusual entity was performed.


2015 ◽  
Vol 81 (5) ◽  
pp. AB414
Author(s):  
Deepak K. Bhasin ◽  
Surinder S. Rana ◽  
Ravi Sharma ◽  
Vishal Sharma ◽  
Rajesh Gupta

1988 ◽  
Vol 69 (4) ◽  
pp. 599-603 ◽  
Author(s):  
Stephen R. Marano ◽  
Peter C. Johnson ◽  
Robert F. Spetzler

✓ A case of recurrent Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) in a child is described with a summary of the clinical presentation and associated malformations, and a review of other cases reported in the literature. The histological examination and electron microscopic findings, with special reference to the cytological changes found during evaluation of the recurrence, are presented. Theories regarding the pathogenesis of Lhermitte-Duclos disease are reviewed.


2021 ◽  
pp. 10-11
Author(s):  
Md. Quamar Zubair ◽  
A. K. Jha Suman

Gall bladder Carcinoma is one of the most common malignancy of the biliary tract. It's indolent and nonspecic clinical presentation and little occurance of pathognomonic radiological features, often hinders the diagnosis at an early stage. Most of the cases are diagnosed incidentally among patients underwent cholecystectomy. Gallbladder Carcinoma is a highly lethal disease, with only 10% patients presenting at a stage amenable to surgical resection. In this study we report incidental carcinoma of gallbladder in patients undergoing cholecystectomy in department of general surgery, Anugrah Narayan Magadh Medical College and Hospital, Gaya, Bihar over a period extending from December 2017 to June 2020.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
A. Hochart ◽  
C. Thumerelle ◽  
L. Petyt ◽  
C. Mordacq ◽  
A. Deschildre

Lipoid pneumonia in children is a rare disorder due to accumulation of fatty oily material in the alveoli and usually associated with an underlying condition. In absence of obvious context, diagnosis remains difficult with nonspecific clinical and radiological features. We report the first case of voluntary chronic aspiration of olive oil responsible for exogenous lipoid pneumonia, in a previously healthy 9-year-old boy. Clinical presentation was atypical; LP was revealed by isolated chest pain. We discuss radiological and bronchial alveolar lavage characteristics suggestive of lipoid pneumonia.Conclusion. Lipoid pneumonia is a disease to be reminded of in children, which can occur with original findings in terms of etiology and clinical presentation.


2001 ◽  
Vol 10 (5) ◽  
pp. 1-9 ◽  
Author(s):  
Tim E. Darsaut ◽  
Giuseppe Lanzino ◽  
M. Beatriz Lopes ◽  
Steven Newman

The term “orbital tumors” comprises a wide variety of lesions that often share the same cardinal clinical finding (exophthalmos) and clinical history. Age at presentation, associated ophthalmological findings, and radiological features, however, provide invaluable information as to the possible histological type of tumor. The present article serves as an introductory overview regarding the pathological characteristics, clinical features, radiological characteristics, and principles of treatment of orbital tumors.


Neurosurgery ◽  
1983 ◽  
Vol 12 (3) ◽  
pp. 303-305 ◽  
Author(s):  
Laligam N. Sekhar ◽  
Peter J. Jannetta

Abstract In a series of 12 cases of thoracic disc herniation operated upon at the University of Pittsburgh, 4 different operative approaches were used. These included laminectomy in 2 early cases, posterolateral extrapleural operation in 5 cases, transthoracic operation in 3 cases, and transpedicular operation in 2 cases. The relative merits of the various approaches are discussed in this paper. The clinical presentation, radiological features, and follow-up data are also presented. Precise preoperative radiological diagnosis was essential in planning the operative strategy. The posterolateral and transpedicular approaches were both satisfactory, but the former had some advantages over the latter. With a mean follow-up period of 5 years, 5 patients were cured, 5 were improved, and 1 was unchanged. One patient was worse due to coexistent amyotrophic lateral sclerosis.


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