Tommaso Tartaglione

Value of Combined Approach With Thallium-201 Single-Photon Emission Computed Tomography and Epstein-Barr Virus DNA Polymerase Chain Reaction in CSF for the Diagnosis of AIDS-Related Primary CNS Lymphoma

PURPOSE To determine the diagnostic capability of thallium-201 (201Tl) single-photon emission computed tomography (SPECT) combined with Epstein-Barr virus DNA (EBV-DNA) in CSF for the diagnosis of AIDS-related primary CNS lymphoma (PCNSL). PATIENTS AND METHODS All human immunodeficiency virus (HIV)-infected patients with focal brain lesions observed between June 1996 and March 1998 underwent lumbar puncture and 201Tl SPECT. Each CSF sample was tested with polymerase chain reaction (PCR) for EBV-DNA. RESULTS Thirty-one patients were included, 13 with PCNSL and 18 with nontumor disorders. In 11 PCNSL patients, EBV-DNA was positive. Thallium-201 uptake ranged from 1.90 to 4.07 in PCNSL cases (mean, 2.77; 95% confidence interval [CI], 2.35 to 3.19) and from 0.91 to 3.38 in nontumor patients (mean, 1.62; 95% CI, 1.30 to 1.94) (P<.0002). Using a lesion/background ratio of 1.95 as cutoff, a negative SPECT was found in one PCNSL case and 16 nonneoplastic cases. A cryptococcoma and a tuberculoma showed highly increased 201Tl uptake. Epstein-Barr virus DNA was never detected in nonneoplastic patients. For PCNSL diagnosis, hyperactive lesions showed 92% sensitivity and 94% negative predictive value (NPV), whereas positive EBV-DNA had 100% specificity and 100% positive predictive value. The presence of increased uptake and/or positive EBV-DNA had 100% sensitivity and 100% NPV. CONCLUSION Combined SPECT and EBV-DNA showed a very high diagnostic accuracy for AIDS-related PCNSL. Because PCNSL likelihood is extremely high in patients with hyperactive lesions and positive EBV-DNA, brain biopsy could be avoided, and patients could promptly undergo radiotherapy or multimodal therapy. On the contrary, in patients showing hypoactive lesions with negative EBV-DNA, empiric anti-Toxoplasma therapy is indicated. In patients with discordant SPECT/PCR results, brain biopsy seems to be advisable.

Early Thalamic Injury Associated with Epilepsy and Continuous Spike–Wave during Slow Sleep

Summary:  Purpose: Mechanisms inducing continuous spike–wave during slow sleep (CSWS) in encephalopathy with electrical status epilepticus during sleep are still unclear. Recently, some sporadic cases with early thalamic injury associated with CSWS have been reported. The aim of the study was to investigate in a population of patients with an early thalamic injury the presence of an activation of paroxysmal activities during sleep, their characteristics, and possible relations to neuroimaging and neuropsychological features.

Early hemispherectomy in catastrophic epilepsy: A neuro-cognitive and epileptic long-term follow-up

The authors report their experience about a neuro-cognitive and epileptic long-term follow-up of children with catastrophic epilepsy treated with hemispherectomy in the first 5 years of life. Nineteen children with resistant epilepsy that significantly interfered with their neuro-cognitive development underwent hemispherectomy within 5 years of life (mean: 2 years, 3 months; range: 5 months to 5 years). All patients were assessed before surgery and after, at least at the end of the follow-up (mean: 6 years and 6 months; range: 2-11 years and 2 months) with a full clinical examination including motor ability and functional status evaluation as well as behaviour observation, neuroimaging and an ictal/interictal prolonged scalp video-EEG. A seizure-free outcome was obtained in 73.7% of patients. Gross motility generally improved and cognitive competence did not worsen, with an evident progress in two cases. Consistently with previous reports, evolution was worse in cortical dysplasia than in progressive or acquired vascular cerebropathies. The excellent epileptic outcome and the lack of developmental deterioration in comparison with other more aged series seem to suggest a possible better evolution in earlier surgery treatment. To confirm this suggestion, however, further experience with larger series is needed.

Response to cidofovir after failure of antiretroviral therapy alone in AIDS-associated progressive multifocal leukoencephalopathy

Progressive multifocal leukoencephalopathy (PML) is a demyelinating brain disorder caused by the polyoma JC virus (JCV). It occurs in approximately 4% of patients with AIDS, producing a rapidly progressive, usually fatal course. There is no proven effective therapy for this condition. Despite initially promising reports of cytosine arabinoside treatment, later studies showed no efficacy of this agent.1,2 Disease stabilization was reported in patients taking highly active antiretroviral therapy (HAART), but further observations did not improve survival.3,4 There is a single anecdotal report of stabilization after cidofovir and cytarabine.5 Cidofovir is the most potent inhibitor of human and murine polyomavirus replication in vitro, and short-term stabilization of PML has been described using this agent alone.6 We report a patient with AIDS-associated PML showing a marked clinical and neuroradiologic improvement after cidofovir was added to HAART. A 32-year-old HIV-positive woman, a former IV drug abuser, was diagnosed with PML after presenting with a paresis …

Preliminary clinical observations on a new trigeminal reflex: The trigemino-cervical reflex

Short latency trigemino-cervical reflexes can be recorded from sternocleidomastoid muscle after stimulation of the infraorbital branch of the trigeminal nerve. We studied the trigemino-cervical reflexes and the conventional blink reflex in three patients with an isolated lesion in the medulla oblongata, eight patients with multiple sclerosis, and two patients with supratentorial ischemic lesion. The trigemino-cervical response was abnormal in the patients with an isolated lesion in the medulla oblongata and in all multiple sclerosis patients, whereas both components of the blink reflex were preserved in the patients with a lesion in the medulla oblongata and in half of the patients with multiple sclerosis. The trigemino-cervical reflex was preserved in patients with supratentorial lesions, whereas the late component of the blink reflex was abnormal. These findings suggest that central pathways generating the trigemino-cervical reflex are confined to the medulla oblongata and that they are independent from those generating the long latency (R2) component of the blink reflex. The trigemino-cervical reflex may help in disclosing and localizing brainstem lesions. NEUROLOGY 1996,46 479-485

Magnetic resonance imaging findings in sudden sensorineural hearing loss.

OBJECTIVE To investigate the role of magnetic resonance imaging (MRI) in the diagnosis of sudden sensorineural hearing loss (SSNHL). METHODS Fifty-four consecutive patients affected by SSNHL were investigated using brain MRI. MRI was performed with an eight-channel phased-array head coil to study the entire audiovestibular pathway and the whole brain. The protocol study consisted of a high-resolution study of the temporal bone, internal auditory canal (IAC), cerebellopontine angle (CPA), and brainstem combining 2 mm thin-slice axial T(2)-weighted two-dimensional fast spin echo (FSE) and fluid-attenuated inversion recovery (FLAIR) sequences, pre- and postcontrast (gadolinium-diethylenetriamine pentaacetic acid) administration fat-suppressed axial T(1)-weighted two-dimensional FSE sequences, and a T(2)*-weighted three-dimensional Fourier transformation-constructive interference in steady state sequence (FT-CISS) , with 0.4 mm ultrathin partitions. The rest of the brain was studied with a 4 mm axial T(2)-weighted FLAIR sequence. RESULTS Thirty-one of 54 (57%) cases of SSNHL presented with MRI abnormalities. In 6 of 54 cases, the detected abnormality was directly correlated to the clinical picture (2 labyrinthine hemorrhage, 1 cochlear inflammation, 1 acoustic neuroma, 1 arachnoid cyst of the CPA, and 1 case of white matter lesions in the pons, compatible with demyelinating plaques along the central audiovestibular nervous pathway, as the first expression of multiple sclerosis). CONCLUSIONS An extensive MRI study of the audiovestibular nervous pathway and of the whole brain, pre- and postparamagnetic contrast administration, is recommended to rule out the wide spectrum of abnormalities that can cause SSNHL.

Acute Necrotizing Encephalopathy during Novel Influenza A (H1N1) Virus Infection

A novel swine‐origin influenza A (H1N1) virus was recently identified in Mexico. Some cases of infection with neurological complications have been reported to date. We report a case of acute necrotizing encephalopathy associated with the novel H1N1 virus in a 2‐year‐old European girl who suddenly developed fever, seizures, and altered mental status. Brain and spinal cord magnetic resonance imaging showed bilateral symmetrical lesions of the insulae, thalami, geniculate bodies, and pons tegmentum suggestive of an acute necrotizing encephalopathy. An involvement of meninges and spinal cord was observed configuring an acute necrotizing meningoencephalomyelitis. ANN NEUROL 2010;68:111–114

Computed tomography (CT) virtual autopsy and classical autopsy discrepancies: Radiologist's error or a demonstration of post-mortem multi-detector computed tomography (MDCT) limitation?

Modern imaging technologies, such as computed tomography (CT) techniques, represent a great challenge in forensic pathology. The field of forensics has experienced a rapid increase in the use of these new techniques to support investigations on critical cases, as indicated by the implementation of CT scanning by different forensic institutions worldwide. Advances in CT imaging techniques over the past few decades have finally led some authors to propose that virtual autopsy, a radiological method applied to post-mortem analysis, is a reliable alternative to traditional autopsy, at least in certain cases. The authors investigate the occurrence and the causes of errors and mistakes in diagnostic imaging applied to virtual autopsy. A case of suicide by a gunshot wound was submitted to full-body CT scanning before autopsy. We compared the first examination of sectional images with the autopsy findings and found a preliminary misdiagnosis in detecting a peritoneal lesion by gunshot wound that was due to radiologists error. Then we discuss a new emerging issue related to the risk of diagnostic failure in virtual autopsy due to radiologists error that is similar to what occurs in clinical radiology practice.

The role of brain infarcts and hippocampal atrophy in subcortical ischaemic vascular dementia

Abstract.We investigated if, in patients with vascular lesions, the variable that best discriminated demented from non–demented patients was the severity of the vascular pathology or the degree of hippocampal atrophy. A total of 39 patients multiple subcortical infarcts, who could be considered as possible vascular dementia with small vessel pathology, with underwent a neuropsychological study and brain magnetic resonance imaging (MRI) DSM IV criteria supported by neuropsychological data were used to distinguish demented from non–demented patients. The MRI study took into account the degree of hippocampal atrophy (hippocampal height and interuncal distance) and the severity of vascular pathology (number of brain infarcts). The distribution of lesions and a factor analysis showed that hippocampal atrophy is a better predictor of dementia than the number of brain infarcts. Multiple subcortical infarcts alone are probably not able to cause clinical dementia but the presence of vascular lesions increases the expression of concomitant Alzheimer’s disease.

Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus: sudden recovery of CSWS

PURPOSE To try to prove in patients with refractory symptomatic epilepsy due to early brain injury involving thalamus and complicated by CSWS the effects of the isolation of the injured hemisphere, performed with functional hemisperectomy, on epilepsy, namely on CSWS. METHODS Full clinical follow-up before and after surgery of two cases with CSWS onset at four years in whom functional hemispherecomy was performed with resection of inter-hemispheric connections. RESULTS An immediate effectiveness of the surgical treatment was observed on both epileptic evolution (no more seizures) and EEG abnormalities. In particular, CSWS completely disappeared, together with a concurrent progressive improving of the cognitive and behavioural disorders. DISCUSSION The isolation of the injured hemisphere through the section of inter-hemispheric cortico-cortical connections could prevent the contralateral diffusion of discharges coming from the injured cortex and cortico-thalamic network, favouring a normal function of thalamo-cortico-thalamic circuitries in the healthy hemisphere. That could explain the disappearance of CSWS after surgery in our patients and the consequent improvement of cognitive abilities and behaviour.