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.

A 15-year-old boy underwent neurological and ophthalmological evaluation. At birth, a severe bilateral microphthalmia, micropenis, and scrotal hypoplasia were diagnosed. Ophthalmologic examination showed right anophthalmia and severe left microphthalmia. Radiological examination showed normal orbital and skull structures. Magnetic resonance imaging (MRI), revealed the absence of the right eye, left microphthalmia, optic nerve hypoplasia, aplasia of the optic chiasm, and tracts. Audiometric examination and electroencephalogram were normal. There was no mental retardation. The chromosomal examination was normal. The patient is examination was also negative for any type of known risk factors

Posterior Reversible Encephalopathy Syndrome (PRES) is a neurotoxic state accompanied by a unique brain imaging pattern. This cliniconeuroradiological entity usually present with visual disturbances (cortical blindness, homonymous hemianopia, visual neglect, blurred vision) along with neurotoxic manifestations. Only a few cases of PRES have previously been reported in patients with advanced HIV disease. The authors describe a case of Posterior Reversible Encephalopathy Syndrome (PRES) in a in patient with advanced HIV /TBC infection who developed a neurotoxic state following TB and ART therapy initiation. They present a comprehensive review of the literature and discuss the pathogenetic hypotheses.

Purpose: To report a case of Wernicke’s encephalopathy in a nonalcoholic woman with secondary hypoadrenalism. Methods: A 58-year-old Italian woman developed Wernicke’s syndrome secondary to recurrent vomiting due to secondary hypoadrenalism. Results: Recurrent vomiting and resulting malnutrition caused a depletion of the patient’s body stores of thiamine and the development of mental confusion and an oculomotor deficit. On the diagnostic suspicion of encephalitis in this patient with immunosuppression due to prolonged cortisone-based therapy, she underwent magnetic resonance imaging (MRI), which showed typical bilateral abnormal lesions pathognomonic of Wernicke’s encephalopathy. The patient improved after parenteral administration of vitamin B1. Conclusions: Wernicke’s encephalopathy must be regarded as a medical emergency that should be suspected in all cases presenting with a sudden deficit or lack of coordination of the ocular movements associated with ataxia and general degeneration of the mental faculties, in patients who have suffered recurrent vomiting or malnutrition for different reasons.

Brain atrophy is a known marker of irreversible tissue damage in multiple sclerosis (MS). Cerebrospinal fluid (CSF) osteopontin (OPN) and neurofilament light chain (NF-L) have been proposed as candidate surrogate markers of inflammatory and neurodegenerative processes in MS.

Amyotrophic lateral sclerosis (ALS) has heterogeneous clinical features that could be translated into specific patterns of brain atrophy. In the current study we have evaluated the relationship between different clinical expressions of classical ALS and measurements of brain cortical thickness. Cortical thickness analysis was conducted from 3D-MRI using FreeSurfer software in 29 ALS patients and 20 healthy controls. We explored three clinical traits of the disease, subdividing the patients into two groups for each of them: the bulbar or spinal onset, the higher or lower upper motor neuron burden, the faster or slower disease progression. We used both a whole brain vertex-wise analysis and a ROI analysis on primary motor areas. ALS patients showed cortical thinning in bilateral precentral gyrus, bilateral middle frontal gyrus, right superior temporal gyrus and right occipital cortex. ALS patients with higher upper motor neuron burden showed a significant cortical thinning in the right precentral gyrus and in other frontal extra-motor areas, compared to healthy controls. ALS patients with spinal onset showed a significant cortical thinning in the right precentral gyrus and paracentral lobule, compared to healthy controls. ALS patients with faster progressive disease showed a significant cortical thinning in widespread bilateral frontal and temporal areas, including the bilateral precentral gyrus, compared to healthy controls. Focusing on the primary motor areas, the ROI analysis revealed that the mean cortical thickness values were significantly reduced in ALS patients with higher upper motor neuron burden, spinal onset and faster disease progression related to healthy controls. In conclusion, the thickness of primary motor cortex could be a useful surrogate marker of upper motor neuron involvement in ALS; also our results suggest that cortical thinning in motor and non motor areas seem to reflect the clinical heterogeneity of the disease.

Acutely ruptured brain arteriovenous malformations (AVMs) are a known etiology of hemorrhagic stroke during pregnancy. The aim of this paper is to report two cases of patients which presented with ruptured AVMs during pregnancy and were successfully treated with endovascular techniques. Peculiar issues related to the application of this treatment strategy in this category of patients will be discussed as well. To the best of our knowledge, this therapeutic approach in cerebral AVMs ruptured during pregnancy has not been described yet.

Recently, neuroimaging studies were performed using 1H-magnetic resonance spectroscopy (1H-MRS), revealing a quantitative alteration of neurochemicals (such as neurotransmitters and metabolites) in several brain regions of patients with autism spectrum disorder (ASD). The involvement of the frontal lobe in the neurobiology of ASD has long been documented in the literature. Therefore, the aim of this study was to analyze the alterations of N-acetylaspartate/creatine (NAA/Cr) and choline/Cr (Cho/Cr) ratios in the frontal lobe subcortical white matter (WM) in ASD patients, in order to reveal any alteration of metabolites that might be the expression of specific clinical features of the disorder.

Lead poisoning is a medical condition caused by increased levels of the heavy metal lead in the body, generally resulting from environmental exposure. Lead poisoning can cause a variety of symptoms which vary depending on the individual and the duration of lead exposure; lead poisoning is more noxious to children than adults, because it can damage the brain and nerve development of children.We report a case of lead poisoning in a 5-year-old girl caused by accidental ingestion of a metal ring. Simultaneously, the child showed anemia, encephalopathy, and peripheral neuropathy with albuminocytological dissociation mimicking Guillain-Barré syndrome.This case suggests that anemia with signs of peripheral and central nervous system damage could be considered as a possible manifestation of lead poisoning in children.

BACKGROUND AND OBJECTIVE: giant pituitary adenomas (GPAs) are benign tumours with a diameter ≥ 4 cm [1]. They can cause symptoms and signs due to the possible hyper-secretion of one or more pituitary hormones, and involvement of the surrounding structures whereas the compression of the pituitary itself can lead to hypopituitarism. METHODS: we report on a young woman with acromegaly due to an inoperable giant GH-secreting pituitary adenoma extending to right cavernous sinus, right orbital cavity, ethmoid, right maxillary sinus, sphenoid sinus, clivus and right temporal fossa, in which medical treatment with Octreotide-LAR was able to promptly relieve headache and bilateral hemianopsia due to optic chiasm involvement, improve acromegaly symptoms and, over the time, control tumor expansion, improving fertility and therefore allowing the patient to become pregnant. RESULTS: Octreotide-LAR therapy was withdrawn during pregnancy and the patient did not experience complications and gave birth to a healthy son. On Magnetic Resonance the size of the tumor at the end of pregnancy and in the subsequent follow up was not increased. CONCLUSION: the history we report, therefore, confirms previous experiences reporting a possible favourable outcome of pregnancy in patients affected by acromegaly and add further information about the behaviour of giant pituitary tumors in patients underwent pregnancy.

BACKGROUND: Thromboembolism is a complication of acute lymphoblastic leukemia therapy in children. The majority of thromboembolic events are cerebral thromboses and deep venous thromboses; many asymptomatic deep venous thromboses are detected in children with acute lymphoblastic leukemia by instrumental screening. The aim of this study was to assess the incidence of asymptomatic cerebral thromboembolic events in children with acute lymphoblastic leukemia (ALL) screened by magnetic resonance imaging and magnetic resonance venography. METHODS: 46 children with acute lymphoblastic leukemia, during the induction phase of the AIEOP ALL 2000 protocol, were stratified into 2 groups. In group "A" cerebral thromboembolic events were suspected following the appearance of suggestive signs and symptoms and confirmed by cerebral magnetic resonance imaging and magnetic resonance venography; in group "B" children underwent a screening by cerebral magnetic resonance imaging and magnetic resonance venography, at set times, in absence of symptoms. RESULTS: We observed one cerebral thromboembolic event in both groups; we found no differences between early detecting asymptomatic cerebral thromboembolic events among monitored and not monitored patients. CONCLUSIONS: Our study does not seem to suggest a screening for asymptomatic cerebral thromboembolic events in children with ALL during the induction phase.

Epidemiological evidence suggests a strict correlation between sudden sensorineural hearing loss (SSNHL) and cerebrovascular disorders. Leukoaraiosis represents a diffuse alteration of the periventricular and subcortical white matter. The aim of our study was to verify if the presence of white matter hyperintensity (WMH) was higher in patients affected by SSNHL compared to controls and evaluate the correlation between WMH and the cardiovascular risk factors, hearing level, and the response to therapy in SSNHL patients. The study group included 36 subjects affected by unilateral SSNHL. Thirty-six age- and sex-matched normal subjects with a negative history of SSNHL were used as controls. All patients underwent magnetic resonance imaging (MRI) (1.5 Tesla GE Signa) and the extent of leukoaraiosis was assessed with the Fazekas scale. The results of the present study demonstrate a high prevalence of WMH in SSNHL patients compared to controls confirming the hypothesis of a vascular impairment in SSNHL patients. The higher recovery rate in patients with greater periventricular white matter hyperintensity (PWMH) may suggest a vascular etiology that is still responsive to medical treatment. We aim to expand both the number of patients and the controls to avoid the limitation of the still small number to warrant solid scientific conclusions.

BACKGROUND: Ophthalmoplegic migraine (OM) is a rare condition characterized by the association of headaches and an oculomotor nerve palsy. The third cranial nerve is commonly involved in recurrent attacks, whereas involvement of the sixth and fourth nerves is uncommon. It is still debated whether an uncontrolled migraine or an oculomotor neuropathy may be the primary cause of ophthalmoplegic migraine. CASES: We report two patients affected by OM with normal magnetic resonance imaging findings and a history of uncontrolled migraine before an attack of OM. CONCLUSION: The cases reported allow us to hypothesize that OM may be considered a form of migraine rather than a cranial neuralgia. It is possible that different factors such as inflammatory or structural factors, may represent a vulnerability of the nerve during a severe migraine attack causing ophthalmoplegia.

Abstract 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.

Prenatal Ultrasonagraphy (US) is the mainstay modality to diagnose fetal abnormalities especially in early pregnancy. Fetal Magnetic Resonance Imaging (MRI) is a useful tool to confirm and to characterize a pathology that is suspected on US, especially in the detection of central nervous system pathologies.The use of ultrafast imaging tecniques gives additional importantant informations and optimal imaging quality, despite fetal motion, in clinical practice. Diffusion Weighted Imaging (DWI), Diffusion Tensor Imaging (DTI), MR Spetroscopy and Functional studies have potential applications in the fetal brain imaging. Fetal MRI could recognize, in contradistinction to US, the development of fetal brain, the multilayered appearance of the cerebral parenchyma, the timing of sulci development and the myelination. The most common indications for fetal MRI are ventriculomegaly, midline anomalies, malformations of cerebral cortical development, posterior fossa anomalies, suspected haemorraghic-ischemic lesions, tumors. Fetal MRI is a safe and powerful complement to US for clinical management and prognostication.

Although the neurohypophyseal hormones vasopressin and oxytocin are mostly known for their role respectively in antiduresis the former and in labour, lactation and maternal behavior the latter, both might exert widespread influences either on emotion and cognition in healthy subjects, showing some gender-related differences. They interact one each other facilitating shifts between positive socially- oriented and defensive states. In fact, vasopressin amplifies the reactivity to stressors showing also beneficial effects on attention, verbal learning as well as memory, whereas oxytocin reduces the amplitude of the stress response, improves emotion processing, and can play a negative effect on memory and verbal learning in healthy individuals. Several data indicate the possible involvement of this neuropeptides in the pathophysiology of psychiatric conditions involving social interactions, such as autism, as well as in schizophrenia and depression. Aim of this paper is to review the literature dealing with the role played by neurohypophyseal hormones in neuropsychiatric disorders.

STUDY DESIGN: Prospective observational study. OBJECTIVE: To assess (1) the evolution of vertebral bone marrow edema (VBME) in patients with A1 vertebral compression fractures (VCFs) conservatively treated and (2) the relationship between VBME and clinical symptoms, evaluated as Visual Analogue Scale (VAS) back pain and Oswestry Disability Index (ODI). SUMMARY OF BACKGROUND DATA: VBME is a marker of acute-subacute vertebral fractures. Little is known about the evolution of VBME in conservatively managed VCFs, as well as its clinical meaning. METHODS: 82 thoracic or lumbar VCFs (21 post-traumatic; 61 osteoporotic VCFs), type A1 according to the AOSpine thoracolumbar spine injury classification system, in 80 patients were treated with C35 hyperextension brace for 3 months, bed rest for the first 25 days. Patients with osteoporotic fractures also received antiresorptive therapy and vitamin D supplementation. At 0 (T0), 30 (T1), 60 (T2), and 90 (T3) days, patients underwent magnetic resonance imaging evaluation and clinical evaluation, using VAS for pain and ODI.The paired t test was used to compare changes within groups at each follow-up versus baseline. The unpaired t test after ANOVA (analysis of variance) was used to compare the 2 groups at each follow-up.The association between VBME area, VAS score, and ODI score was analyzed by the Pearson correlation test. The tests were 2-tailed with a confidence level of 5%. RESULTS: A significant VBME mean area, VAS, and ODI scores reduction was recorded at 60 and 90-days follow-ups versus baseline. A positive correlation between VBME reduction and clinical symptoms improvement (VAS and ODI scores improvement) was found in both traumatic and osteoporotic VCFs. CONCLUSION: In benign A1 VCFs conservatively managed, VBME slowly decreases in the first 3 months of magnetic resonance imaging follow-up. This VBME reduction is related to clinical symptoms improvement.

OBJECTIVE: To assess (1) the evolution of VBME in patients with A1 VCFs conservatively treated and (2) the relationship between VBME and clinical symptoms, evaluated as VAS (Visual Analogue Scale) Back Pain and ODI (Oswestry Disability Index). SUMMARY OF BACKGROUND DATA: Vertebral Bone Marrow Edema (VBME) is a marker of acute/sub-acute vertebral fractures. Little is known about the evolution of VBME in conservatively managed vertebral compression fractures (VCFs), as well as its clinical meaning. METHODS: 82 thoracic or lumbar VCF (21 post-traumatic; 61 osteoporotic VCFs), type A1 according to AOSpine Thoracolumbar Spine Injury Classification System, in 80 patients were treated with C35 hyperextension brace for three months, bed rest for the first twenty-five days. Patients with osteoporotic fractures also received Antiresorptive therapy and vitamin D supplementation. At 0 (T0), 30 (T1), 60 (T2) and 90(T3) days, patients underwent MRI evaluation and clinical evaluation, using VAS for Pain and ODI.The paired t test was used to compare changes within groups at each follow-up versus baseline. The unpaired t test after ANOVA was used to compare the two groups at each follow-up.The association between VBME area, VAS score, ODI score was analyzed by the Pearson Correlation test. The tests were two-tailed with a confidence level of 5%. RESULTS: A significant VBME mean area, VAS and ODI scores reduction was recorded at 60- and 90-days follow-ups versus baseline. A positive correlation between VBME reduction and clinical symptoms improvement (VAS and ODI scores improvement) was found both in traumatic and osteoporotic VCFs. CONCLUSION: In benign A1 VCFs conservatively managed, VBME slowly decreases in the first 3 months of MRI follow-up. This VBME reduction is related to clinical symptoms improvement.