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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dysplastic gangliocytoma is defined as a rare cerebellar mass lesion with malformative, hamartomatous and neoplastic features, arising from cerebellar neurons and affecting the population in early adulthood. Common clinical presentation is in the form of headache, features of raised intracranial tension or hydrocephalus. It may also be found as an incidental lesion. Even though many cases of this disease entity have been reported in literature, its association with syrinx is less commonly highlighted and hence we are reporting these two cases which we encountered at our institute. Presence of an associated syrinx should not deter one to make a preoperative diagnosis of this condition once the striking imaging findings are visualized.
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PMID:Lhermitte-duclos disease with syringomyelia. A report of two cases. 2429 73

Cerebellar gangliocytoma can correspond to Lhermitte-Duclos disease, a benign hamartomatous malformation encountered in young adults. It can also be a part of gangliogliomas/gangliocytomas family, which usually encompasses temporal pediatric neoplasms associated with longstanding seizures. We report a case of a young 11-year-old patient who presented with a gangliocytoma of the cerebellum revealed by neurologic manifestations (headache, dyspraxia, equilibrium and gait disturbances). Diagnosis was made on surgical material. Tumour was characterized by dysplastic mature ganglion cells, perivascular lymphocytic infiltrates and no glial neoplastic component. By immunohistochemistry, ganglion cells expressed neurofilaments, MAP2 protein, synaptophysin, chromogranin A and S100 protein. BRAF V600E mutation was absent. Clinical characteristics, radiology, histopathology of the two main diagnoses are discussed.
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PMID:[Cerebellar gangliocytoma in an 11-year-old child]. 2549 64

We report a case of a 60-year-old man who presented with weight gain, headaches, dizziness, erectile dysfunction and decreased libido. He was found to have elevated adrenocorticotropic hormone (ACTH) and prolactin serum levels. The imaging studies revealed a 1.4 cm sella/suprasellar mass which was compressing the optic chiasm. Histologic slides of the lesion showed a pituitary adenoma, marked by a proliferation of biphenotypic appearing cells, associated with a gangliocytoma, and marked by a proliferation of atypical appearing neuronal cells arranged against a glial-appearing background. Pituitary adenoma-gangliocytomas are benign combination tumors that rarely occur in the sellar region. Adenomas in this setting are sometimes functional, and rare patients with mixed adenomas (adenomas secreting more than one hormone) have been reported. To our knowledge, there has been only one other report of a combined ACTH and prolactin-producing adenoma with gangliocytoma, reported in a patient who also had acromegaly. In our patient, the immunohistochemical stains demonstrated that the bulk of the adenoma cells stained with prolactin antibody, and scattered clusters of cells within the adenoma stained positively for ACTH. The adenoma did not stain with antibodies to any of the other anterior pituitary hormones. Postoperatively, the elevated prolactin and ACTH levels returned to normal levels and there was no evidence of residual tumor. Adequate sampling and immunohistochemistry are important in rendering a correct diagnosis and in identifying the hormone status of mixed adenoma-gangliocytomas.
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PMID:Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma. 2631 58

Lhermitte-Duclos disease (LDD), a disorder first described by French physicians Lhermitte and Duclos in 1920 [25], is a benign, slow growing dysplastic gangliocytoma of the cerebellum, characterized by replacement of the granule cell layer by abnormal granule and Purkinje like cells. The most frequent presenting signs and symptoms are megalocephaly, increased intracranial pressure, nausea, hydrocephalus, ataxia, gait abnormalities, and intermittent headaches, all of which are attributed to the mass effect [6,11,25]. Many cases are associated with a mutation in the phosphatase and tensin homolog or PTEN gene which is also involved in numerous otherwise unrelated central nervous system abnormalities, namely Cowden syndrome [1,6,11], autism spectrum disorder [18], cerebral cortical dysplasia [11,30] and Bannayan-Riley-Ruvalcaba syndrome [30]. The presence of cortical heterotopia has been reported in a small number of LDD cases [3,5,17,32]. We describe a unique case of LDD with cerebral cortical heterotopic grey matter containing neurofibrillary tangles.
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PMID:Lhermitte-Duclos disease with neurofibrillary tangles in heterotopic cerebral grey matter. 2754 76

Tacrolimus is a widely used macrolide immunosuppressant in transplant surgery, with mild and major neurologic side effects. A 21-year-old woman had undergone preemptive transplantation of a kidney from her mother. On the 1st postoperative day, the patient had headache, nausea, vomiting, and agitation. Magnetic resonance imaging (MRI) of the brain showed hyperintensity and a lesion in the right mesial temporal lobe. After we switched from tacrolimus to cyclosporine, the symptoms regressed. Persistence of the lesion, confirmed by repeated MRI, required that the patient be operated on. Pathologic examination showed the gangliocytoma, a rare brain tumor. Our case shows that preexisting brain lesions may cause tacrolimus-induced neurotoxicity in the early postoperative period.
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PMID:Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report. 2793 67

Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. It is probably hamartomatous, although the exact pathogenesis remains unknown. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). It typically presents in young adults, although it has been encountered at all ages. We present the case of bilateral cerebellar location of this pathology in a 50-year-old man presented with a progressive onset and worsening of headaches accompanied by nuchal rigidity, photophobia and nausea awakening each morning. Upon physical examination, the patient was awake with a discrete right vestibular syndrome made of positive Romberg without nystagmus. Magnetic Resonance Imaging (MRI) was performed and revealed salient "tiger stripe" appearance of the bilateral cerebellar cortex relevant to a Lhermitte-Duclos disease.
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PMID:The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology. 3148 96

Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare benign cerebellar tumor that is typically observed but may occasionally become symptomatic and requires surgical intervention. The condition is caused by a mutation in the phosphatase and tensin homolog (PTEN) gene, which results in dysregulation of the mammalian target of rapamycin pathway. A germline PTEN mutation results in multi-organ involvement and is termed Cowden syndrome. There is a scarcity of surgical videos in the published literature that demonstrate an intraoperative resection of this lesion and illustrate the pathology in Vivo. We present an operative video of a surgical resection of a symptomatic dysplastic cerebellar gangliocytoma in a 44-yr-old male patient who presented with a 3-mo history of progressive headaches and hydrocephalus. The patient underwent an endoscopic third ventriculostomy and, subsequently, a right suboccipital craniotomy for microsurgical resection of the mass. The procedure was performed with the patient in the lateral position. The microscope was positioned at the head of the bed and the stereotaxic system monitor on the patient's left side. The patient tolerated the procedure well and imaging obtained at 18 mo was negative for residual or recurrent disease. The patient gave written consent for video recording as part of the surgery informed consent. No identifiable images or video footage of the face are shown, and institutional review board approval was deemed unnecessary.
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PMID:Resection of a Symptomatic Dysplastic Cerebellar Gangliocytoma: 2-Dimensional Operative Video. 3180 81

BACKGROUND Lhermitte-Duclos disease (LDD) is caused by a rare slow-growing mass in the cerebellum. LDD generally is experienced by young adults, but also it has been encountered in the pediatric population. Lhermitte and Duclos first described cerebellar dysplastic gangliocytoma in 1920. The first case they described included occipital headache, paroxysmal vertigo, falls, hearing problems, and memory deficits. Our patient had typical symptoms of the disorder such as headache, nausea, vomiting, blurred vision, and imbalance. The purpose of this case report was to describe the outcome of a computerized dynamic posturography (CDP) vestibular training program combined with home-based exercises designed to improve balance function and reduce the risk of falling by an individual with a posterior fossa tumor. CASE REPORT A 36-year-old male patient was diagnosed with dysplastic gangliocytoma/ganglioglioma according to magnetic resonance imaging, computed tomography, and pathology reports on March 28, 2016. The patient was treated by partial cerebellar tumor resection on April 7, 2016. After the operation, he reported severe imbalance, nausea, and vomiting for 1 month and visited the Audiology Department on October 20, 2016. The patient was evaluated with the CDP-sensory organization test (SOT) and his composite equilibrium score of this examination was 48, 31% below normal. We administered a 6-week posturography-assisted vestibular rehabilitation (VR) protocol (extending an hour per week) combined with a home-based exercise program twice in 2 years. In the second evaluation we applied in 2018, SOT composite equilibrium score increased to 72 after VR, reaching normal limits. After 2 years, his complaints slightly alleviated and his SOT scores were better when we compared the VR results in 2016. CONCLUSIONS We demonstrated that long-term VR may affect a patient with dysplastic cerebellar gangliocytoma (LDD) presenting imbalance or dizziness.
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PMID:Role of Long-Term Vestibular Rehabilitation in a Patient with Posterior Fossa Tumor: A Case Report with 2 Years of Follow-Up. 3291 76

Paraneoplastic neurologic syndromes are a rare and heterogeneous group of immune-mediated syndromes caused by underlying solid and nonsolid tumors. We present a case of 8-year-old female with long history of mild headaches and central instability who presented multiple poorly defined signal abnormalities at the subcortical white matter of both cerebral hemispheres and cerebellar atrophy on brain magnetic resonance imaging. Further studies revealed a posterior mediastinum ganglioneuroma derived from a mature ganglioneuroblastoma that was treated with surgery. Two paraneoplastic neurologic syndromes were considered: Anti-N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis due to the resolution of subcortical signal abnormalities after mediastinal mass resection and opsoclonus-myoclonus-ataxia syndrome due to cerebellar atrophy. Intertnational guideline established the criteria for definite diagnosis of paraneoplastic neurologic syndromes and detection of onconeural antibodies is not mandatory for their diagnosis. Paraneoplastic neurologic syndromes may appear several years before the tumor is detected.
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PMID:Brain paraneoplastic syndromes in a patient with mediastinal ganglioneuroma. 3294

The following case report features a middle-aged female patient, previously diagnosed with Cowden syndrome, who presented to the hospital with symptoms of headaches and changes in vision that began with no apparent cause and persisted for almost a month. MRI of the head confirmed a diagnosis of dysplastic cerebellar gangliocytoma, also known as Lhermitte-Duclos disease. This cerebellar tumor, while extremely rare in incidence, is classified as the most common type of brain lesion in adult patients with Cowden syndrome. This report will also include a comprehensive literature review of Cowden syndrome and Lhermitte-Duclos disease, with greater emphasis on the radiologic characteristics of Lhermitte-Duclos disease.
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PMID:Radiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome: A Case Study and Literature Review. 3308 15


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