Michele Palma

Posterior Reversible Encephalopathy Syndrome Associated With Methotrexate Neurotoxicity: Conventional Magnetic Resonance and Diffusion-Weighted Imaging Findings

The addition of intrathecal methotrexate to treatment protocols has increased survival rates in children with acute lymphoblastic leukemia but is also associated with varying degrees of neurotoxicity. We describe a 15-year-old female patient diagnosed with acute lymphoblastic leukemia presenting with status epilepticus after receiving intrathecal methotrexate. Magnetic resonance imaging showed reversible cortical and subcortical changes consisting of high-intensity lesions on T2-weighted and fluid-attenuated inversion recovery sequences with postgadolinium enhancement, low signal intensity on diffusion-weighted imaging and increased apparent diffusion coefficient. These findings were consistent with the posterior reversible encephalopathy syndrome. We report our conventional magnetic resonance and diffusion-weighted imaging findings and briefly discuss the pathophysiology of the syndrome.

Very early onset and greater vulnerability in schizophrenia: A clinical and neuroimaging study.

Although schizophrenia has been diagnosed in children, this group of disorders has received too little attention in the clinical and research literature. Preliminary data suggest that early onset schizophrenia (EOS) and very early onset schizophrenia (VEOS) tend to have a worse outcome than adult onset schizophrenia, and seem to be related to a greater familial vulnerability, due to genetic, psychosocial, and environmental factors. Recently, advanced neuroimaging techniques have revealed structural and functional brain abnormalities in some cerebral areas. This paper reports on a case diagnosed as VEOS, with premorbid year-long psychopathological history. The patient showed atypical proton magnetic resonance spectroscopy findings, and normal brain and spine computer tomography and brain magnetic resonance images.

Primary intracranial dural B cell small lymphocytic lymphoma

Primary central nervous system non-Hodgkin lymphomas (PCNSNHL) are extranodal lymphomas arising within and confined to the central nervous system (CNS). A frequency of 1 – 3% of all nonHodgkin lymphomas and 2 – 4% of all brain neoplasms has been reported, with a particularly high incidence in the AIDS population, in transplant recipients and immunocompromised patients [1 – 3]. Meningeal involvement by malignant lymphomas is most often a manifestation of diffuse aggressive lymphomas [1 – 3] while primary dural lymphomas with no associated cerebral parenchyma lesions are rare diseases [4 – 22]. We describe a case of primary B cell small lymphocytic lymphoma (SLL) involving the dura of the frontoparietal and temporal regions, and include a review of the literature, performing a Medline search for cases of primary intracranial dural lymphoma [4 – 22]. A 44-year-old immunocompetent woman referred a 2-week history of headache, diplopia, blurring of vision, loss of concentration, and right eye exophthalmos. Cranial magnetic resonance imaging (MRI) disclosed a large right dural thickening frontal mass, which was hyperintense in T1 weighted images and hypointense in T2 sequences. The administration of Gadolinium diethylenetriamine pentaacetic acid (Gd DTPA) revealed a wide contrast enhanced area involving the meninges and initial invasion of the underlying brain on the right frontoparietal and sphenoidal regions [Figure 1(A)]. Ophthalmic examination confirmed right eye exophthalmos and diplopia; fundoscopic evaluation showed mild pupil pallor, but no evidence of edema. Lumbar puncture was performed: cerebrospinal fluid (CFS) did not show malignant cells; CSF biochemical evaluations were within normal range with a glucose level of 65 mg/dl (n.v. 40 – 70 mg/dl) and protein level of 40 mg/dl (n.v. 15 – 45 mg/dl); polymerase chain reaction studies on CSF samples for Varicella Zoster, Herpes Simplex, Epstein-Barr viruses were negative. The patient underwent a right frontoparietal craniotomy. Intraoperatively, a diffuse plaque-like mass firmly adherent to the dura and infiltrating the pia mater and arachnoid membrane was encountered. The lesion could not be excised, and biopsies were performed. Histological examination revealed a monomorphic population of small lymphocytes diffusely infiltrating the dura [Figure 2(A)]. Immunohistochemical analysis demonstrated CD20 [Figure 2(B)], CD5 [Figure 2(C)], and CD23 [Figure 2(D)] positivity, bcl2 protein expression, and lambda light chain restriction, suggesting a typical pattern of SLL. CT scans of the neck, chest, abdomen, and pelvis showed no evidence of disease. Routine laboratory tests were within normal range, including serum lactate dehydrogenase 92 U/l (n.v. 100 – 190 U/l) and b2 microglobulin 1 mg/ml (n.v. 0.8 – 2.0 mg/ml). Cytological and immunophenotypical analysis did not show bone marrow and peripheral blood involvement. Serum HIV, EBV, CMV tests were negative. The patient was treated according to the IELSG 20 protocol and treatment consisted of four cycles of high dose i.v. methotrexate

Acute extrapyramidal disorder with bilateral reversible basal ganglia lesions in a diabetic uremic patient: Diffusion-weighted imaging and spectroscopy findings

Acute movement disorders associated with bilateral lesions in the basal ganglia are increasingly described in patients affected by diabetes and uremia. Pathophysiology has not been utterly understood yet, but it is likely to be multifactorial, with both ischemic/microvascular and metabolic/toxic factors determining the lesions and symptoms. We have studied a uremic diabetic patient who was admitted in emergency after presenting choreic movements, in which CT and MR, including diffusion-weighted imaging and spectroscopy, showed bilateral symmetric basal ganglia lesions with regression at follow-up. This is the first report in the literature describing spectroscopic findings in this condition.

Cerebrovascular Disorders in the Prenatal Period

Fetal stroke can occur between the 14th week of pregnancy and the beginning of labor. The incidence is approximately 17-35 of 100 000 live births. Risk factors are correlated to the mother, the pregnancy, or the factors associated with the fetus itself. Computerized tomography and magnetic resonance imaging are the most commonly used imaging techniques. The authors studied 3 cases with neurological symptoms by magnetic resonance imaging and magnetic resonance-angiography. In these cases, the authors found massive involvement of the left hemisphere that was presumptively correlated with the persistence of the oval foramen. Magnetic resonance-angiography showed a flow reduction in the left cerebral vessels, and in 1 case, there was also persistence of the fetal primitive trigeminal artery. The discrepancy between the extent of the cerebral lesions and the neurological symptoms is associated with cerebral plasticity, which is greater in damage occurring early in fetal life.

Os odontoideum as a rare but possible complication in children with dyskinetic cerebral palsy: a clinical and neuroradiologic study.

The authors describe a 12-year-old boy with dyskinetic (athetoid-dystonic subtype) cerebral palsy and os odontoideum. Dystonic and choreoathetotic components in cerebral palsy are movement disorders that are difficult to treat and cause major disability. Dystonic posturing causes excessive flexion, extension, and rotation of the neck. Repetitive abnormal movements in patients with this type of cerebral palsy give rise to a higher incidence of pathologic conditions affecting the craniovertebral junction. Os odontoideum is one of these pathologies, and it represents a rare anomaly of the odontoid process. There are only a few reports describing os odontoideum in children with dyskinetic cerebral palsy. This clinical and neuroradiologic study focuses on the problem of atlantoaxial instability and os odontoideum in these forms of cerebral palsy, which is too often underestimated.

Description of Six Cases of Child Cauda Equina Leptomeningitis: An Emerging Disease

Child cauda equina leptomeningitis (CCEL) is a typical clinical example of aseptic meningitis with patterns of an emerging disease, and it affects children aged 2–9. Here we will describe six cases of CCEL. After the prodromes, all children underwent an acute phase with hypoasthenia of the lower limbs, hyporeflexia, staggering and ataxia with steppage. Only in one case there were generalized fits and coma of grade 1–2 too. All children underwent a spinal Magnetic Resonance Imaging (MRI), proving pathologic enhancement of cauda equina and conus medullaris leptomeningitis. At the same time, MRI made possible the differential diagnosis between cauda equina leptomeningitis and isolated minor forms of Guillain-Barre syndrome involving the lower limbs. Three hypotheses will be formulated for understanding the pathogen mechanism(s) of CCEL. The first one is based on the presence of an immediate viral damage on the meninges, the second one, the more likely, contemplates the occurrence of an immunomediated mechanism in a host genetically prone to react in an abnormal way from an immune viewpoint. The third hyphotesis consists in a two-time damage: an early immediate damage from the virus, and a later immunomediated reaction.

Costello syndrome: cognitive and proton magnetic resonance spectroscopy findings--a case report.

The authors describe a girl with Costello syndrome who showed cerebral palsy and neurosensorial deafness. Brain computer tomography and magnetic resonance findings were normal. Multivoxel proton magnetic resonance spectroscopy showed a lowering of the peak of choline with a reduced choline/creatine ratio at the level of the centrum semiovale. These findings might be due to a congenital dysmyelinating or hypomyelinating condition. A complete neuroimaging study can play a relevant role to better clarify the pathogenesis of brain involvement in Costello syndrome.