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

We report a case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum), an uncommon disorder of uncertain pathogenesis characterized by disarrangement of the normal cerebellar laminar cytoarchitecture. A 40-year-old man was admitted because of vomiting and syncope of a few days' duration, and a 2-month history of intermittent headaches and unsteady gait. A computed tomographic scan of the patient's head showed obstructive hydrocephalus due to displacement of the fourth ventricle by a large, nonenhancing cerebellar mass. The magnetic resonance images of the brain also revealed a space-occupying lesion within the right cerebellum with unusual septation. After surgery, the histologic examination confirmed the diagnosis of Lhermitte-Duclos disease. This is the first report of Lhermitte-Duclos disease in Taiwan.
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PMID:Lhermitte-Duclos disease: first report in Taiwan. 979 36

The March COM: A 16 year old female presented with headaches and cerebellar dysfunction. MR images showed a mass lesion of the right cerebellar hemisphere with mass effect on the medulla. The mass exhibited a striated pattern of alternating isointense and hypointense zones on T1-weighted images that did not contrast enhance. The lesion was hyperintense on T2-weighted images, and also showed a striated appearance. A suboccipital craniotomy and resection of the lesion was performed. Microscopically, the specimen consisted of widened folia and a disorganized cerebellar architectonic pattern in which the internal granular cell layer was occupied by a population of large dysmorphic nerve cell bodies. Patient's diagnosed with Lhermitte-Duclos disease must be adequately evaluated for Cowden's syndrome.
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PMID:March 2000: A 16 year old female with a cerebellar mass. 1141 81

Lhermitte-Duclos disease is a rare abnormality occurring in the cerebellum with only 130 cases reported in the literature. There is debate as to whether this abnormality is a hamartoma, a malformation or a tumour. In this case report we discuss the spectroscopy findings from two patients presenting with this disease. The patients, one 40-year-old Caucasian woman with a 6-year history of headaches, unsteady gait and falls, deterioration in vision and another 28-year-old Caucasian man with a 1-year history of headaches and a previous history of a transient stroke, were found to have this lesion in the cerebellum. Proton spectroscopic data were obtained using a single-voxel PRESS technique (TE=135 ms, TR=1600 ms), from the region of the abnormality. The results were expressed as ratios under the three prominent resonances representing choline (Cho), creatine (Cr), and N-acetyl (NA) moieties. The metabolite ratios were compared to normative data. The two cases demonstrated reduced ratios in NA/Cho and NA/Cr in relation to the controls. The ratios of Cho/Cr appeared closer to the normal mean ratio. There were peaks attributable to lactate in both cases. The low NA/Cr and NA/Cho ratios could be due to the apparent lack of neuronal architecture and the presence of embryonic neural tissue, which does not express NA, indicating more favourably towards a 'benign' hamartoma rather than a tumour.
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PMID:MR imaging and spectroscopy in Lhermitte-Duclos disease. 1510 36

Lhermitte-Duclos disease is a rare clinical entity characterized by slow deformation of the cerebellar lesion. A 53-year-old woman presented with Lherimitte-Duclos disease manifesting as ataxic gait, occipital headache, and loss of consciousness. Magnetic resonance imaging demonstrated striated and laminar pattern lesions in the right cerebellar hemisphere and vermis. She underwent subtotal removal of the vermis compressing the brainstem, but the cerebellar hemisphere appeared normal and was preserved. Histological findings were consistent with Lherimitte-Duclos disease. Two years later, magnetic resonance imaging revealed enlargement of the right hemispheric lesion. Intraoperative appearance of the hemisphere demonstrated marked enlargement of the folia. Subtotal removal was performed. Histological examination showed no malignant findings. Lhermitte-Duclos disease may recur, and should be carefully followed up as a low-grade neoplasm, not as a hamartomatous disorder.
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PMID:Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report. 1809 41

A 45-year-old man presented with a headache. The medical history included a partial thyroidectomy, a colon polypectomy, and stomach tumors (all benign adenomas) 34, 2, and 1 year earlier, respectively. CT showed a space-occupying lesion in the left cerebellar hemisphere with calcification. The mass showed a striated pattern of hyperintensity on T2-weighted images. An FDG-PET scan was performed after the intravenous administration of 250 MBq (6.8 mCi) F-18 FDG. The images of the brain showed a hypermetabolic area in the left cerebellar hemisphere. The whole body images showed no abnormal accumulation other than physiologic uptake. A presumptive diagnosis of Lhermitte-Duclos disease (LDD) was made.
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PMID:FDG PET in Lhermitte-Duclos disease. 1809 62

The authors report the case of a 7-year-old boy with a history of developmental delay who presented with aggressive behavior. A magnetic resonance (MR) image showed a mass lesion originating from the cerebellar vermis with an atypical folial pattern and contrast enhancement. Histologically, the subtotally resected specimen consisted mostly of neuropil with nodular foci of ganglion cells. Lhermitte-Duclos disease (LDD) was diagnosed in the patient. A retrospective review of the tissue sections showed a nidus of associated astrocytic proliferation, suggesting a diagnosis of ganglioglioma. Five years later, the patient experienced an altered mental state and a facial droop. An MR image revealed a cerebellar mass with cystic areas and an enhancing nodule. The resected tissue specimen consisted primarily of a mixed proliferation of glial and ganglion cells consistent with a ganglioglioma. Two years later, a third craniectomy was performed in the patient for worsening headache and ataxia. Histologically, the tumor showed progressive anaplasia and was most accurately classified as an anaplastic ganglioglioma. Immunohistochemically, most of the tumor cells were immunoreactive for anti-phospho-mammalian target of rapamycin (mTOR) and phospho-S6 ribosomal protein antibodies. In contrast, the subpopulation of neoplastic ganglion cells in the tissue, particularly from the first surgery, did not express phosphatase and tensin homolog deleted from chromosome 10 (PTEN). This immunohistochemical pattern suggests that the large dysplastic ganglion cells (the gangliocytomatous component) forming the greater part of the lesion were associated with activation of the phosphatidylinositol 3-kinase-PTEN/Akt/mTOR signaling pathway, a feature previously reported in LDD. This case represents the first report of an anaplastic ganglioglioma arising in an LDD-like lesion.
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PMID:Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. 1845 85

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.
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PMID:Isolated cerebellar Rosai-Dorfman granuloma mimicking Lhermitte-Duclos disease. Case report. 1964 43

Lhermitte-Duclos disease (LDD) is a pathologic entity with progrediating, diffuse hypertrophy chiefly of the stratum granulosum of the cerebellum. Typically LDD is a unilateral lesion of the cerebellum or in vermis. Here we report a case of LDD with bilateral lesions of cerebellar hemispheres managed surgically. A 28-year-old woman presented with one-year history of progressive headache, nausea, vomiting, and blurred vision. Neurologic examination revealed a bilateral mild papilledema, mild dysmetria, and dysdiadochokinesia. The cerebellar lesions caused moderate mass effect in posterior fossa with hydrocephalus, and Chiari type I malformation. We performed the suboccipital-retrosigmoid approach, and removed completely the left intracerebellar mass. Symptoms related to elevated intracranial pressure disappeared in a short period postoperatively.
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PMID:Bilateral Lhermitte-Duclos disease. 2050 58

Lhermitte-Duclos disease (LDD) is a rare cerebellar disorder characterized by diffuse or focal enlargement of cerebellar folia. Clinical manifestations are usually related to a mass effect and secondary obstructive hydrocephalus. Increased intracranial pressure symptoms and cerebellar symptoms are the most frequent patient complaints. We describe the case of a patient with LDD who developed secondary obstructive hydrocephalus. A 68-year-old woman was brought to the emergency room for sudden vertigo following several bouts of vomiting and headache. There were no external signs of trauma, serious illness or infection. On admission, the patient was alert and had no neurological deficits. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed hydrocephalus and a cerebellar mass in the right cerebellar hemisphere compressing the fourth ventricle. Suboccipital craniotomy and subtotal removal of the mass was performed. Pathological study of the surgical specimen showed abnormal ganglionic neurons and an enlarged molecular layer compatible with dysplastic gangliocytoma. Cytoreduction can achieve improvement in symptoms caused by mass effect, but postoperative swelling may aggravate obstructive hydrocephalus. Therefore, if symptoms still remain after removal of the mass, an additional shunting procedure may be needed as a further management option.
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PMID:Lhermitte-Duclos disease presenting with hydrocephalus. 2211 44

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


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