Diffuse Leptomeningeal Enhancement Research Articles

Primary leptomeningeal oligodendroglioma with documented progression to anaplasia and t(1;19)(q10;p10) in a child

Oligodendrogliomas are infiltrating gliomas typically arising in the cerebral cortex and white matter. Rare examples present with diffuse leptomeningeal involvement in the absence of a clinically/radiologically detectable intraparenchymal tumor [1–5, 7, 8, 10, 14, 15]. Although oligodendrogliomas are rare in children, most primary leptomeningeal oligodendrogliomas have occurred in the first decade of life (Table 1). We report the case of a 3-year-old girl, who presented with progressive regression of her ability to walk and an increase of cranial circumference. MRI showed marked hydrocephalus and diffuse leptomeningeal enhancement without supratentorial intraparenchymal lesions (Fig. 1a–c). A C3–C4 mildly enhancing 5 mm nodule was present, which could not be defined with certainty as meningeal or intraparenchymal (Fig. 1c). Cerebrospinal fluid (CSF) was xanthochromic, with high protein (177 mg/dl), normal glucose (63 mg/dl) and increased white blood cells (47 cells/mm, 50% mononuclear). Bacterial cultures were negative, but polymerase chain reaction showed CSF positivity for M. tuberculosis. A diagnosis of basal meningitis was made and the child was unsuccessfully treated with anti-tuberculosis therapy. When a meningeal biopsy demonstrated a low-grade neuroectodermal tumor consistent with low-grade oligodendroglioma, the patient was started on chemotherapy, according to the SIOP protocol for low-grade gliomas, based on vincristine and carboplatin, changed later to vincristine and cisplatin alternating with cyclophosphamide every 6 weeks due to an allergic reaction to carboplatin. Chemotherapy was suspended at 18 months, after remarkable improvement of her clinical condition. She also received radiotherapy at the level of C2–C4, because of the gradual increase of the cervical nodule (54 Gy in 30 fractions). The child remained stable for the following 3 years, when her tumor progressed to anaplastic oligodendroglioma, as confirmed by a new biopsy of a left frontal paraventricular nodule. At this time, MRIs demonstrated multiple enhancing nodules filling the ventricles, suggesting involvement of the sub-ependymal parenchyma (Fig. 1d–f). The patient received temozolomide, but her clinical condition continued to deteriorate and she expired, 7 years from the original diagnosis. The first biopsy consisted of a very small fragment of leptomeninges showing dominant reactive changes with hyperplastic meningothelial cells and inflammation. A small focus of monomorphous cells with round nuclei diffusely expressing S100 protein and focally GFAP was seen (Fig. 2a). The second biopsy revealed a highly cellular neoplasm with small cells with hyperchromatic round nuclei and perinuclear halos similar to the original ones (Fig. 2b). Numerous mitoses were identified (up to 8/10 HPF). S100 protein expression was extensive, GFAP was focal. Neu-N, synaptophysin, EMA and CKAE1/AE3 stains were negative. p53 was expressed in rare nuclei. MIB-1 labeling index was high (up to 50%). Electron microscopy demonstrated round cells with scant organelles lacking ependymal features. 1p/19q co-deletion and S. Rossi A. P. Dei Tos F. D. Paola M. Bendini S. Agostini P. Longatti Departments of Pathology, Neuroradiology, Pediatrics and Neurosurgery, Regional Hospital, Treviso, Italy

32 Vertigo with hearing loss as the first symptom of leptomeningeal carcinomatosis originating from colorectal carcinoma

Introduction Leptomeninges are common dissemination site of advanced malignant disease. The most frequent primary sites are breast, lung, melanoma and stomach, whereas colon is rarely reported. Case report We report a case of a 70- year-old man who was admitted to the neurology department with a three-week history of mild frontal headache, vertigo and vomiting. The patient had no history of malignancies or any other serious diseases. The neurological examination showed an ataxic gate and hearing loss on the right ear. Findings of the multi-slice computed tomography (MSCT) of the brain were unremarkable. Magnetic resonance imaging (MRI) of the brain showed multiple periventricular white matter lesions. During the next few days the patient symptoms progressed to include right peripheral facial nerve palsy, complete hearing loss and mental alteration. Body temperature and inflammation parameters were normal all of this time. Gadolinium-enhanced MRI was performed and revealed diffuse leptomeningeal enhancement of the cranial base a thickening of both of the vestibulocochlear nerves especially right one. Cerebrospinal fluid (CSF) analysis showed sterile hypercellular (1184/3mm3) CSF with predominantly low-differentiated malignant cells with numerous mitoses (Figure 1), hypoglycorrhachia (1, 7 mmol/L) and elevated protein level. The highly increased serum levels of the tumor marker carbohydrate antigen 19-9 was present. Ultrasound and MSCT of the abdomen, esophagogastroduodenoscopy as well as ultrasound of the thyroid gland and chest X-ray did not find any signs of malignant disease. However, colonoscopy revealed an infiltrating process that prominates to the lumen of the ascending colon. The patient showed no symptoms of intestinal obstruction or any other GI tract symptoms. Histopatological analysis of biopsy samples verified adenocarcinoma. The patient refused intratecal chemotherapy and underwent whole-brain radiotherapy. Despite treatment, the patient’s state deteriorated and as a result he died shortly after. Conclusion Unilateral hearing loss progressing to bilateral deafness within short period of time is a rare clinical manifestation of the leptomeningeal carinomatosis.

Leptomeningeal dissemination of low-grade intramedullary gliomas: About one case and review

Leptomeningeal (LM) dissemination arises rarely in patients with low-grade gliomas. Few cases of LM dissemination of spinal cord low-grade gliomas have been reported in children. Here, we report a case of an intramedullary spinal cord low-grade glioma occurring in an adult presenting by LM dissemination. A 57-year-old man presented with a history of headaches and visual acuity deterioration. Neurological examination revealed mental confusion, decreasing left limb strength and left positive Babinski sign. Lumbar puncture showed low CSF glucose, high CSF protein and no tumor cells. Spinal MRI revealed an intramedullary spinal lesion with a diffuse leptomeningeal enhancement. A spinal meningeal biopsy showed arachnoiditis with isolated atypical cells which were cytokeratin immunopositive. A differential diagnosis was proposed between arachnoiditis close to an infectious process or close to meningeal carcinomatosis. An intramedullary biopsy was programmed but the patient died 1 month after biopsy. The post-mortem examination showed thickened leptomeninges and an ill-circumscribed intramedullary grey lesion. Histological examination revealed a low-grade glial neoplasm composed of monomorphous spindle “piloid” cells with diffuse dissemination to leptomeninges. Glial fibrillary acidic protein immunostaining showed scattered immunoreactive cells. Clinical and pathological features of this case are unusual. Pathogenesis and prognosis of low-grade gliomas with LM dissemination are poorly understood. This case, like cases occurring in children, cannot easily be classified in the present WHO system of classification of CNS tumors. Spinal low-grade gliomas with LM dissemination may represent a possible distinctive clinico-pathological entity.

Primary diffuse leptomeningeal gliomatosis

A 20-year-old woman with a history of seizures presented symptoms of walking difficulties for the past six months. Clinical examination was suggestive of a craniovertebral junction anomaly. A cerebrospinal fluid study showed mild protein elevation with no evidence of an infective pathology. Craniospinal MRI revealed diffuse nodular leptomeningeal enhancement of the brain and spinal cord. Histopathological examination was suggestive of a low-grade glioma, and the patient was diagnosed with primary diffuse leptomeningeal gliomatosis. So far, the patient has survived for more than 110 months without aggressive therapy.

A new Thr49Pro transthyretin gene mutation associated with leptomeningeal amyloidosis

Leptomeningeal amyloidosis is a rare central nervous system manifestation of systemic amyloidosis from transthyretin (TTR) mutation. Ten TTR gene mutations associated with this condition have been described. We report the clinical, radiological, and pathological features of a case of leptomeningeal amyloidosis from a novel Thr49Pro TTR gene mutation. A 53 year-old man presented with recurrent episodes of transient aphasia and right hand stiffness, headaches, and peripheral neuropathy. A surgical biopsy of the leptomeninges and superficial cortex, and DNA sequences of the TTR gene were analyzed. Elevated cerebrospinal fluid (CSF) protein and diffuse leptomeningeal enhancement of the neuraxis from the cortical sulci to the cauda equina on contrast MRI were the defining clinical features. A surgical biopsy revealed TTR amyloid deposition in the subpia and within the leptomeningeal blood vessels. DNA sequencing showed a proline-for-threonine substitution at codon 49 of the TTR gene in one allele. A novel Thr49Pro TTR gene mutation is associated with leptomeningeal amyloidosis resulting in recurrent CNS symptoms.

Primary diffuse leptomeningeal gliomatosis in a 2-year-old girl

Background Primary diffuse leptomeningeal gliomatosis is a rare tumor disease affecting the leptomeninx of the CNS. It is thought to originate from subarachnoidal glial cell nests. The symptoms are often rather unspecific. Surgery is not an option and despite the use of chemotherapy and radiotherapy patients rarely survive for more than 12 months. Case Description We present a case of PDLG in a 2-year-old girl, the youngest patient reported on so far. She presented with increasing somnolence, intermittent strabism, and vomiting. Magnetic resonance imaging of the cranium showed enlarged ventricles and contrast enhancement of the basal leptomeninx. Cerebrospinal fluid diagnostic studies showed a mild pleocytosis and elevated protein levels but no tumor cells or evidence of infection. A ventriculoperitoneal shunt was placed and a biopsy of the leptomeninx was taken in the right Sylvian fissure. The histopathology findings suggested a nonspecific meningeal inflammation. Because the girl developed spinal symptoms with paresthesia and hyperalgesia, a spinal MRI was performed which showed a similar contrast enhancement of the spinal leptomeninx. A spinal biopsy was taken and subsequently a paucicellular astrocytic tumor was diagnosed corresponding to a WHO I diffuse leptomeningeal gliomatosis. Chemotherapy with vincristine, carboplatin, and etoposide was initiated (Protocol SIOP-LGG 2004) but was stopped by the parents when the child was in partial remission after 50 weeks because of a neurologic deterioration. The girl has so far survived for more than 29 months. Conclusion Primary diffuse leptomeningeal gliomatosis must be included in the differential diagnosis of diffuse leptomeningeal contrast enhancement in young children. There are promising treatment options that need to be carefully evaluated.

Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy–Walker complex following ventriculoperitoneal shunt placement for hydrocephalus

Neurocutaneous melanosis (NM) coexisting with the Dandy-Walker complex (DWC) is a rare condition, with fewer than 15 cases reported in the literature. The authors present a case of an infant with NM and DWC suffering from progressive brainstem compression following ventriculoperitoneal (VP) shunt placement for hydrocephalus. This 1-year-old boy with congenital melanocytic nevi had met normal developmental milestones until the age of 11 months, when he began regressing in ambulation and language function. Intractable vomiting had developed 1 week later. Magnetic resonance (MR) imaging of the brain revealed DWC with hydrocephalus, and spinal MR images demonstrated a proliferative process within the meninges, consistent with NM. The patient underwent right frontal VP shunt placement resulting in immediate symptom relief, but 3 weeks later became irritable, increasingly lethargic, unable to pull to stand, and unable to tolerate solid food without choking. Due to these symptoms and intractable vomiting, the patient presented to the authors' institution. Brain MR imaging revealed a new-onset diffuse cystic process with anterior and posterior brainstem compression, marked kinking of the cervicomedullary junction, melanocyte pigmentation of the left temporal lobe, diffuse leptomeningeal enhancement, and no evidence of hydrocephalus. Consistent with these imaging findings, the degree of brainstem involvement upon gross visualization predictably deterred resection attempts beyond those necessary for biopsy. Pathological examination revealed diffuse melanocytosis, and the family decided not to pursue aggressive measures postoperatively. This report indicates the potential for rapid intracranial manifestation of diffuse melanocytosis in NM patients. Although the prognosis is poor, early neurosurgical involvement in these patients may provide tissue diagnosis and the potential for decompression if the process is caught early in its course.

NEUROCUTANEOUS MELANOSIS ASSOCIATED WITH AUTOIMMUNE DIABETES MELLITUS

### Case report. A 12-year-old boy with multiple nevi presented with 6 months of polydipsia (>4 L/day), polyuria, and a 40-pound weight loss that progressed over weeks to intractable headache and vomiting. The patient subsequently developed ataxia, complex partial seizures, and encephalopathy. Medical history was significant for numerous (40 to 50) benign congenital nevi ranging in diameter from 1 to 10 cm. He did not have a previous diagnosis of neurocutaneous melanosis (NCM) or a history of CNS disease. The initial workup revealed hyperglycemia (random blood glucose of 400) and hemoglobin (Hgb) A1c 11.9%. Anti–glutamic acid decarboxylase (GAD) antibody was 2.1 U/mL (normal <0.1). There was diffuse leptomeningeal enhancement on MRI. Initial lumbar puncture revealed an opening pressure of 40 cm H2O, white blood cell count of 23, glucose 40, and protein 193. Cytology from the CSF demonstrated atypical melanocytes consistent …

Primary diffuse leptomeningeal gliomatosis followed with serial magnetic resonance images

We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) followed up with serial magnetic resonance images (MRI). A 45-year-old man manifested with bilateral abducens nerve palsy and meningisms. Repeated MRI revealed diffuse leptomeningeal enhancement throughout the central nervous system without intra-axial mass accompanied with the dilatation of ventricles and focally enlarged cerebral sulci. Brain biopsies showed a leptomeningeal gliomatosis. The MRI findings described here would contribute to the diagnosis of PDLG among other common diseases diffusely spreading along the leptomeningeal structures.

Primary disseminated leptomeningeal oligodendroglioma with 1p deletion

The authors report the case of a 2-year-old boy with a primary, diffuse leptomeningeal oligodendroglioma in which the deletion of chromosome arm lp was identified by performing a fluorescence in situ hybridization (FISH) analysis. This previously healthy child initially presented with malaise, anorexia, nausea, vomiting, and macrocephaly. Imaging studies confirmed the presence of hydrocephalus, and a ventriculoperitoneal shunt was placed. The postoperative course was complicated by emesis, continued weight loss, and numerous seizurelike episodes. A contrast-enhanced magnetic resonance imaging study performed approximately 10 weeks postoperatively showed diffuse leptomeningeal thickening and enhancement without evidence of an intraparenchymal mass lesion. A right frontal lobe brain biopsy revealed a hypercellular proliferation of small oligodendroglioma-like cells, which occupied the leptomeninges diffusely and spared the underlying cortical gray matter. The tumor cells displayed prominent perinuclear clearing and had evenly spaced, uniformly round nuclei. Occasional mitotic figures were observed. Background vessels were thin and delicate, and there was no evidence of necrosis. The tumor cells showed strong immunoreactivity for S100 protein; the results of immunohistochemical staining were negative for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, NeuN, and synaptophysin. The deletion of lp was demonstrated by FISH analysis; lq, 19p, and 19q were intact. This appears to be the first reported case of a primary diffuse leptomeningeal oligodendroglioma in which a lp deletion was identified.

A case report and review of the literature

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare central nervous system neoplasm in which gliomatous tissue is diffusely identified in the subarachnoid space with no evidence of a primary intraparenchymal tumor. A 52-year-old man presented low back pain followed by sudden unconsciousness and had also cognitive dysfunction and meningeal sign. Examinations of cerebrospinal fluid (CSF) did not show malignant cells but increased protein and pleocytosis. Magnetic resonance (MR) imaging demonstrated diffuse leptomeningeal enhancement without any source of intraparenchymal lesion. Fluid-attenuated inversion recovery (FLAIR) also demonstrated individual diffuse high intensity area in the subarachnoid space. A biopsy disclosed wide spreading of anaplastic glial cells within the leptomeninges. He died 3 months later because of disease progression despite both radiotherapy and chemotherapy. Post-mortem examination identified PDLG and several neuropathological features of glioblastoma as well. Reviewing previous cases of PDLG instructs that this entity is rare, resembles meningitis in clinical pictures, usually occurs in a relatively younger population and has more progressive clinical course than the ordinary form of malignant gliomas.

Leptomeningeal familial amyloidosis:

Sirs: We report on a 31-year old woman with a known history of familial amyloidosis with the transthyretin (TTR) variant Leu12Pro. The pedigree can be traced back to the year 1780. For three years the patient had suffered from a primary partial secondary generalising epilepsy with a constant semiology starting with spitting automatisms, indicating a focus of the non dominant hemisphere [15]. Interestingly, the mother of our patient also suffered from epilepsy with the same semiology and developed severe bilateral visual impairment in her later life. The patient was admitted to our hospital because she became increasingly drowsy and had developed a delusional state at the same time as pneumonia. The patient had received a liver transplant nine months previously and was on a stable immunosuppressive medication (Cyclosporine 100 mg/d, mycophenolate mofetil 1000 mg/ d). Clinically, the patient was drowsy and not well orientated regarding time, place and person. All other findings were completely normal and there were no clinical and electrophysiological signs of peripheral neuropathy. Two days later under antibiotic treatment for pneumonia with cefuroxim the patient showed normal vigilance, was fully orientated but showed slight attention deficits leading to a mini mental score (MMSE) of 27 points. The electroencephalogram showed a diffuse (6 Hz) and focal bitemporal slowing without epileptiform discharges. Cerebrospinal fluid examination revealed a normal opening pressure but xanthochromia with an increased protein level of 229 mg/dl and normal cell count (2/mm), cell cytology, glucose CSF/serum-ratio, and lactate. Tests for oligoclonal bands in the CSF were negative. Magnetic resonance imaging (MRI) showed in the T1 images after contrast administration a diffuse leptomeningeal enhancement around the brainstem, cerebellum and lower parts of the cerebral sulci. No focal intracerebral abnormalities were detected in T2* images. The ophthalmologic examination was completely normal including split lamp investigation and electroretinogram. The MRI and CSF findings together with the known familial amyloidosis indicate a leptomeningeal involvement due to TTR accumulation [12]. This is the second report of a leptomeningeal amyloidosis with a Leu12Pro TTR gene mutation, which was initially described by Brett et al. [5]. Several different TTR gene mutations have been shown to cause this form of amyloidosis (Asp18Gly, Val30Met, Val30Gly, Phe64Ser, Ala36Pro, Gly53Glu, Tyr69His, Ala25Thr, Tyr114Cys) (see [9]). Some of the TTR gene mutations have been associated with both central and peripheral nervous system disorders, whereas others have been characterized mainly by CNS symptoms, including dementia, seizures, ataxia, and stroke-like episodes. By contrast to non–familial forms of amyloidosis, susceptibility irregularities within the cerebral parenchyma were not observed in our patient. The mainstay of therapy in familial amyloidosis is liver transplantation. However, recently it was shown that oculeptomeningeal involvement is not prevented in all patients with initial familial amyloidotic polyneuropathy [2]. In this study, out of 22 patients, three patients developed ocular involvement and two patients exhibited de novo amyloid deposition in the leptomeninges after transplantation, all patients with a Tyr114Cys TTR gene mutation. This observation might be explained by TTR synthesis by the retina and choroid plexus demonstrating the need for additional treatment options like gene therapy [11] or drug interference with the pathogenetic P. P. Urban (&) AE S. Hagele Department of Neurology University of Mainz Langenbeckstr. 1 55101 Mainz, Germany Fax: +49-6131/175-625 E-Mail: urban@neurologie.klinik.uni-mainz

Radiotherapy plus concomitant and adjuvant temozolomide for leptomeningeal pilomyxoid astrocytoma: a case study

Pilomyxoid astrocytoma (PMA) has recently been identified as a new entity among brain tumors in the hypothalamic/chiasmatic region in young children [1]. PMA was previously grouped with pilocytic astrocytoma but has a distinctive monomorphous appearance and generally behaves more aggressively with a higher rate of local recurrence and a substantial rate of leptomeningeal dissemination. Currently, there is no standard of care in treating patients with PMA. In the largest series published to date on 21 cases, four patients have been treated with a combination of radiotherapy and chemotherapy with a recurrence in three after a median follow up of 5 months, however, none of them had leptomeningeal disease at presentation [2]. We report a 33-year-old male with a PMA in the lateral ventricle with leptomeningeal seeding who initially showed a response to a combined regimen of radiotherapy and temozolomide. A 33-year-old right-handed male was admitted to another hospital for headache, nausea, diplopia and disorientation. Cranial CT scan was normal. He improved rapidly and was discharged with a diagnosis of vertebrobasilar migraine. Four weeks later he presented with increasing bifrontal headache, progressive short-term memory deficits, and complex visual hallucinations. Neurologic examination revealed disorientation to time and place, agitation, mild nuchal rigidity, and mild bilateral papilledema. Cranial CT showed slightly enlarged ventricles. A lumbar puncture revealed yellowish cerebrospinal fluid with increased total protein (11 g/l), normal glucose and increased cell count (64 leukocytes/ml). The CSF pressure was elevated (50 cm H2O). Blood pigments were consistent with a subarachnoid bleeding several weeks earlier, but neither MR angiography nor CT angiography showed an aneurysm. Following the lumbar puncture, his headache and cognitive functioning improved. Cranial and spinal MRI with Gadolinium showed a slightly enhancing lesion in the right anterior ventricular horn and diffuse leptomeningeal enhancement. A repeated lumbar puncture yielded atypical cells, suspicious for tumour. He was diagnosed with a ventricular tumour with leptomeningeal seeding that had bled into the subarachnoid space. The ventricular tumour was resected and a ventriculoperitoneal shunt was placed. Pathology was consistent with a PMA (Fig. 1). R. H. Enting (&) Department of Neurology, University Medical Center Groningen, 30001, 9700 RB Groningen, The Netherlands E-mail: r.h.enting@neuro.umcg.nl Fax: +31-50-3611707

Subacute aseptic meningitis as neurological manifestation of primary Sj ögren's syndrome

Sjögren's syndrome (SS) is an autoimmune disease characterized by inflammatory infiltration and secondary chronic dysfunction of exocrine glands. Systemic (extraglandular) manifestations of the disease occur in one-third of the patients, including a wide spectrum of peripheral and central neurological disorders. We report a case of subacute afebrile aseptic meningitis (AM) as neurological manifestation of primary SS. The neurological symptomatology presented gradual onset and progression, including diplopia, mild headache, pain and stiffness of the neck. The clinical examination pointed out xerostomia and xerophthalmia. Diagnosis of SS was confirmed by Schirmer's tear test and histopathology of the labial salivary glands. The neurological involvement was highlighted by gadolinium-enhanced magnetic resonance imaging of the brain which displayed an increased diffuse leptomeningeal enhancement. Cerebrospinal fluid (CSF) analysis showed moderate pleocytosis with prevalence of polymorphonuclear leukocytes and increased protein level but no evidence of Ig intrathecal synthesis. A cycle of intravenous steroid therapy led to a complete disappearance of the neurological symptomatology and to normalization of the CSF inflammatory pattern. Given the unusual presentation of this case of AM, which resembled the characteristics of a chronic meningitis rather than those of an acute form, in patients affected by SS we must stress the importance of cephalic symptoms such as headaches and cervical stiffness (even if mild and without fever) as possible signs of central neurological involvement of the disease.

Vertebral and Spinal Cord Sarcoidosis

Campbell SE, Reed CM, Bui-Mansfield LT, Fillman E 64-year-old woman with a history of sarcoidosis presented with back and abdominal pain and was unresponsive to nonsteroidal antiinflammatory medications. In addition, the patient complained of weakness in her right arm. Physical examination of the abdomen was unremarkable, and examination of the spine revealed normal flexion and extension and no focal tenderness. Neurologic examination revealed slightly decreased strength in the right arm and hand. A posteroanterior chest radiograph showed a bilateral diffuse reticulonodular pulmonary parenchymal pattern, right paratracheal stripe thickening, and bilateral hilar lymphadenopathy (Fig. 1A). CT of the chest confirmed bilateral hilar, right paratracheal, and subcarinal lymphadenopathy and interstitial pulmonary disease. CT also revealed widespread retroperitoneal lymphadenopathy and multiple lytic lesions within the vertebral bodies and posterior elements of the thoracolumbar spine. Subsequent MRI of the spine revealed decreased T1 signal intensity and increased T2 signal intensity scattered throughout the spine (Fig. 1B). After IV sodium gadopentate was administered to the patient, multiple brightly enhancing lesions within the vertebral bodies, pedicles, laminae, and spinous processes were identified. Diffuse leptomeningeal and nerve root enhancement and an enhancing parenchymal spinal cord mass were also present (Fig. 1C). Biopsy of a vertebral lesion revealed noncaseating granuloma consistent with sarcoidosis without evidence of malignancy (Fig. 1D). Gram stains and cultures for fungi, aerobic and anaerobic organisms, and acid-fast bacilli were all negative. The differential diagnosis for diffuse marrow infiltration, enhancing intraaxial spinal cord mass, diffuse meningeal and nerve root enhancement, and mediastinal and retroperitoneal lymphadenopathy includes metastatic disease, lymphoma, leukemia, hematogenous osteomyelitis (tuberculous, fungal), and sarcoidosis [1]. Sarcoidosis is a systemic granulomatous disease of unknown etiology that has been shown to affect nearly every organ system. Osseous involvement occurs in 1–13% of cases, typically in the small tubular bones of the hands and feet [2, 3]. Sarcoidosis may affect either the vertebral column or the spinal cord. Typical vertebral column manifestations are lytic lesions with sclerotic borders that enhance on MRI and enhancement of the intervertebral disk [1, 3–5]. Typical spinal cord manifestations are leptomeningeal enhancement, enhancing spinal cord mass, nerve root “clumping,” and enhancing nerve root [4]. The most common spinal cord manifestation is leptomeningeal disease [4]. Given the variable manifestations of sarcoidosis within the spine, this disease should be considered in the differential diagnosis along with multiple sclerosis [4]; tuberculous meningitis [4]; and widespread vertebral involvement of metastatic disease, infection, or lymphoma [1]. Sarcoidosis is typically treated with corticosteroid therapy, after which clinical symptoms and even MRI findings may completely or partially resolve in 29% of the patients [6]. Prednisone

Metastatic disease of the brain: extra-axial metastases (skull, dura, leptomeningeal) and tumour spread

Extra-axial intracranial metastases may arise through several situations. Hematogenous spread to the meninges is the most frequent cause. Direct extension from contiguous extra-cranial neoplasms, secondary invasion of the meninges by calvarium and skull base metastases, and migration along perineural or perivascular structures are less common. Leptomeningeal invasion gives rise to tumour cell dissemination by the cerebrospinal fluid (CSF), eventually leading to neoplastic coating of brain surfaces. Contrast-enhanced magnetic resonance (MR) imaging is complementary to CSF examinations and can be invaluable, detecting up to 50% of false-negative lumbar punctures. MR findings range from diffuse linear leptomeningeal enhancement to multiple enhancing extra-axial nodules, obstructive communicating and non-communicating hydrocephalus. Both calvarial and epidural metastases infrequently transgress the dura, which acts as a barrier against tumour spread. Radionuclide bone studies are still a valuable screening test to detect bone metastases. With computed tomography (CT) and MR, bone metastases extending intracranially and primary dural metastases show the characteristic biconvex shape, usually associated with brain displacement away from the inner table. Although CT is better in detecting skull base erosion, MR is more sensitive and provides more detailed information about dural involvement. Perineural and perivascular spread from head and neck neoplasms require thin-section contrast-enhanced MR.

Extranodal Rosai-Dorfman Disease Involving the Meninges

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.

Extranodal Rosai-Dorfman Disease Involving the Meninges

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.

Intracranial contrast-enhancing masses in infants with capillary haemangioma of the head and neck: intracranial capillary haemangioma?

Contrast-enhancing intracranial masses are rarely found in infants with extracranial capillary haemangiomas (CH). We aimed to assess their nature and progression in three patients undergoing CT and/or MRI. The changes in size of both extra- and intracranial lesions were recorded. In a fourth case, a single examination was obtained. All patients harboured one or two enhancing intracranial nodular, meningeal-based lesions. Diffuse leptomeningeal enhancement of the cerebellar surface was also seen in one, which disappeared at follow-up. In all but one of the cases, the intracranial lesions were on the same side as the extracranial CH. These lesions and the extracranial CH demonstrated parallel changes in size (suggesting that both represent CH) during follow-up of 1-2 years: the size of intracranial lesions and the extracranial CH decreased in two cases, whereas it was unchanged in the third. One patient had a persistent trigeminal artery, while another had cerebellar atrophy with high signal in the cortex on T2-weighted images. In some cases, extracranial CH are part of PHACE syndrome; the association with intracranial CH might represent a peculiar phenotype of this rare vascular phakomatosis. As extracranial CH are known to regress spontaneously in the majority of cases, a conservative approach is recommended also for presumed intracranial CH; surgery should be avoided unless follow-up studies demonstrate growth.

Diffuse Leptomeningeal Enhancement, ???Ivy Sign,??? in Magnetic Resonance Images of Moyamoya Disease in Childhood

Diffuse Leptomeningeal Enhancement, ???Ivy Sign,??? in Magnetic Resonance Images of Moyamoya Disease in Childhood