Potential benefits of early aggressive immunotherapy in Susac syndrome.

Susac syndrome is a rare vasculopathy of unknown aetiology, affecting prominently young women and electively targeting brain (encephalopathy), retina (visual field defects), and cochlea (hearing loss), of which optimal treatment has yet to be established. We report clinical, CSF and MRI features together with the long-term outcome in a monocentric series of eight consecutive patients with unusual sex ratio (5 male; 3 female), to define the best diagnostic/therapeutic strategy. Six patients presented with the classical clinical triad within less than 6 months after symptoms onset; two did not suffer from sensorineural hearing loss. All but one received a treatment combining high doses of methylprednisolone and cyclophosphamide intravenously. The first two patients had very delayed diagnosis (6-4 months) resulting in severe cognitive sequelae. The third one had only mildly delayed diagnosis (2 months) with subsequent behaviour impairment and severe right hypoacousia. All three were unable to return to work. The last five patients who had early diagnosis and undelayed aggressive treatment were able to resume their professional activities.

Diagnostic and therapeutic pitfalls in neurosarcoidosis.

Neurosarcoidosis is a diagnostic challenge, especially in the absence of systemic involvement, even when cerebral biopsies show noncaseating granulomas. We report a patient with a pineal germinoma associated with a extensive peri- and intra- tumoural granulomatous reaction, who was first diagnosed as possible neurosarcoïdosis. A second patient was initially considered as suffering from Multiple Sclerosis. Brain biopsy showed typical granulomas and gallium scintigraphy revealed other locations of the disease. Unfortunately, he developed a severe, steroid-induced, epidural lipomatosis at the Th3-Th8 levels and died unexpectedly after surgical decompression. Granulomatous inflammation in a tissue obtained by biopsy from a midline lesion should be always considered for the differential diagnosis of germinoma. Corticosteroid-sparing immunosuppressant drugs should be used early in neurosarcoïdosis.

Poliomyelitis-like syndrome with matching magnetic resonance features in a case of Lyme neuroborreliosis.

Lyme disease is a multisystemic disorder caused by an epizootic organism of the spirochete group, called Borrelia burgdorferi, which is transmitted to humans by ticks of the genus Ixodes. Lyme neuroborreliosis may occur during the early dissemination phase, most often as a painful meningo-radiculitis and very rarely as a radiculo-myelitis, whereas encephalomyelitis is observed in the late phase. We report the case of a patient with an early subacute poliomyelitis-like syndrome closely matching the selective involvement of the anterior horns and roots of the cervical spinal cord seen on magnetic resonance imaging. This condition improved with appropriate antibiotics.

Poliomyelitis-like syndrome with matching magnetic resonance features in a case of Lyme neuroborreliosis.

Lyme disease is a multisystemic disorder caused by an epizootic organism of the spirochete group, called Borrelia burgdorferi (Bb), which is transmitted to humans by ticks of the genus Ixodes. Lyme neuroborreliosis may occur during the early dissemination phase, most often as a painful meningo-radiculitis and very rarely as a radiculo-myelitis, whereas encephalomyelitis is observed in the late phase. We report the case of a patient with an early subacute poliomyelitis-like syndrome closely matching the selective involvement of the anterior horns and roots of the cervical spinal cord seen on magnetic resonance (MR) imaging. This condition improved with appropriate antibiotics.

Intraoperative 3T MR imaging for spinal cord tumor resection: feasibility, timing, and image quality using a "twin" MR-operating room suite.

We assessed feasibility, safety, and timing of an original intraoperative MR procedure in 3 cases of resection of spinal cord glioma by using a clinical 3T MR system connected to an adjacent operating room in a design being coined "twin" or "dual" MR-operating room suite.

Acute ischaemic pontine stroke revealing lyme neuroborreliosis in a young adult.

We report the case of a 23-year-old male patient who suddenly developed right hemiparesis, cerebellar ataxia, dysarthria, and bilateral dysmetria. Brain magnetic resonance (MR) examination demonstrated hyperacute ischaemic lesions within the pons. CSF analysis revealed a high protein content, lymphocytic pleocytosis, and oligoclonal IgG bands not present in the serum. Elevated IgM and IgG anti-Borrelia burgdorferi antibodies were shown in both serum and CSF samples, associated with an intrathecal synthesis of these antibodies. Ischaemic CNS lesions have been rarely observed as the first manifestation of Lyme neuroborreliosis. The putative mechanism for parenchymal ischaemia is the local extension of inflammatory changes from meninges to the wall of penetrating arterioles.

L-2-Hydroxyglutaric aciduria: clinical, genetic, and brain MRI characteristics in two adult sisters.

L-2-Hydroxyglutaric (L-2-HG) aciduria is a rare inherited metabolic disease usually observed in children. Patients present a very slowly progressive deterioration with cerebellar ataxia, mild or severe mental retardation, and various other clinical signs including extrapyramidal and pyramidal symptoms, and seizures. The disease is characterized by increased levels of L-2-HG in body fluids such as urine and cerebrospinal fluid. We report on two sisters from consanguineous parents, in whom L-2-HG aciduria was diagnosed at an adult age. Although magnetic resonance imaging and spectroscopic findings were severely abnormal in both, they experienced a different clinical course. The older sister presented with severe mental retardation, recurrent epileptic seizures, and progressive deterioration in her ability to walk and to talk; she is now confined to a wheelchair with severe speech deficit. In contrast, the younger sister only had a few epileptic seizures in childhood and moderate mental retardation, is still able to walk, and performs manual work, and has a social life in a specialized institution for moderately mentally handicapped persons. For the two patients, a complete deletion of exon 9 was demonstrated in a gene located on chromosome 14q22.1, which most probably encodes for L-2-hydroxyglutarate dehydrogenase. The pathological findings observed in this metabolic disorder could therefore be related to a toxic effect of L-2-hydroxyglutarate on the central nervous system, although the presence of other toxic metabolites cannot be excluded.

Usefulness of magnetic resonance-derived quantitative measurements of cerebral blood flow and volume in prediction of infarct growth in hyperacute stroke.

BACKGROUND AND PURPOSE: The identification of the tissue at risk for infarction remains challenging in stroke patients. In this study, we evaluated the value of quantitative cerebral blood flow (CBF) and cerebral blood volume (CBV) measurements in the prediction of infarct growth in hyperacute stroke. METHODS: Fluid-attenuated inversion recovery (FLAIR), diffusion-weighted (DW), and gradient-echo echo-planar perfusion-weighted (PW) sequences were obtained in 66 patients within 6 hours of stroke onset; ischemia was confirmed on follow-up FLAIR images. We delineated the following: (1) the initial infarct on DW images, (2) the area of hemodynamic disturbance on mean transit time (MTT) maps, and (3) the final infarct on follow-up FLAIR images. MTT, CBF, and CBV were calculated in the following areas: area of initial infarct (INF), area of infarct growth (IGR, final minus initial infarct), the hemodynamically disturbed area that remained viable (OLI, hemodynamic disturbance minus final infarct), and all contralateral mirror regions. RESULTS: Compared with mirror regions, the MTT in abnormal areas was always prolonged. The respective mean+/-SD CBF and CBV values were as follows: for INF, 28+/-16 mL/min per 100 g and 6.9+/-2.7%; for IGR, 36+/-20 mL/min per 100 g and 8.9+/-3.1%; for OLI, 50+/-17 mL/min per 100 g and 11.2+/-3%; and for mirror regions, 64+/-23 mL/min per 100 g and 8.7+/-2.5%. The CBV and CBF values were significantly different between all abnormal areas (except for the CBF between INF and IGR). In the area of DW/PW mismatch, a combined CBF or CBV threshold of 35 or 8.2, respectively, predicted evolution to infarction with a sensitivity of 81% and a specificity of 76%. CONCLUSIONS: Quantitative measurements of CBF and CBV in hyperacute stroke may help to predict infarct growth and to select the subjects who will benefit from thrombolysis.

Vasospasm after subarachnoid hemorrhage: diagnosis with MR angiography.

BACKGROUND AND PURPOSE: The possibility of treating intracranial vasospasm has increased the significance of its diagnosis and follow-up; however, so far, no ideal method is available. The goal of this study was to assess the accuracy of MR angiography versus intraarterial angiography (IA-DSA) in detecting vasospasm. METHODS: The study included 42 patients with acute spontaneous subarachnoid hemorrhage (SAH). Serial MR angiograms (minimum, two per patient within 10 days after the event; total, 149) were obtained prospectively using a 3D time-of-flight technique covering the circle of Willis at 0.5 T. Forty-seven MR angiograms could be compared with intraarterial angiograms obtained within 24 hours of MR angiography. Vascular narrowing on both studies was rated consensually by two pairs of neuroradiologists using a scale from 0 (no narrowing) to 3 (severe narrowing). Categories 0 and 1 were considered an absence of vasospasm and categories 2 and 3 a presence of vasospasm. RESULTS: Agreement between MR angiography and IA-DSA (assessed with weighted kappa statistics) was substantial for the middle and anterior cerebral arteries (MCA and ACA) but moderate for the internal carotid artery (ICA). The sensitivity, specificity, accuracy, and positive and negative predictive values of MR angiography for detecting patients with vasospasm were 92%, 98%, 96%, 92%, and 98%, respectively. Considering each vessel separately, specificity was high for all locations (95-99%) and sensitivity was excellent for the ACA (100%) but poorer for the ICA (25%) and MCA (56%). CONCLUSION: MR angiography at 0.5 T is capable of identifying vasospasm after acute SAH but is less sensitive than IA-DSA for depicting vasospasm in the ICA and MCA.

Eosinophilic pleocytosis and myelitis related to Toxocara canis infection.

Toxocara canis causes the visceral larva migrans syndrome in which central nervous involvement is rare. We report the case of a 40-year-old woman presenting with a subacute weakness of the right leg and dysaesthesiae in the right Th8-Th10 dermatomas. Spinal magnetic resonance imaging examination showed abnormal hyperintensity within the spinal cord. Cerebrospinal fluid analysis revealed eosinophilic pleocytosis. Antibody titres to Toxocara canis were higher in the cerebrospinal fluid than in the serum. Treatment using mebendazole led to a complete clinical recovery, normalization of cerebrospinal fluid parameters and improvement in spinal magnetic resonance imaging abnormalities.

Resolution of early diffusion-weighted and FLAIR MRI abnormalities in a patient with TIA.

We report a patient with a clinical history and neurologic examination consistent with acute stroke. Diffusion-weighted and fast fluid-attenuated inversion recovery MRI obtained 4 hours after stroke onset detected focal abnormalities suggestive of acute ischemic brain damage. The neurologic deficit and the imaging abnormalities both resolved completely at follow-up. This patient illustrates complete resolution of early changes observed with diffusion-weighted MRI at the hyperacute phase in a TIA.

MR-monitored remitting-relapsing pattern of cortical involvement in Rasmussen syndrome: comparative evaluation of serial MR and PET/SPECT features.

We report the serial MR and PET/SPECT findings in a 2 1/2-year-old boy presenting with Rasmussen syndrome and highlight the close qualitative correlations between the results of the imaging modality and the functional isotopic techniques. The latter demonstrated a wider field of extension of the disease process. Routine MRI demonstrated its ability to detect brain changes matching the more sensitive PET and SPECT data and correlated well with the clinical evolution.

MRI of cervical cord lesions and their resolution in Toxocara canis myelopathy.

We report serial MRI findings in a 58-year-old man with cervical cord involvement by Toxocara canis, in whom antihelminthic chemotherapy yielded improvement of the neurological deficits and cord lesions seen on MRI.

Whipple disease confined to the central nervous system in childhood.

In a case of pediatric Whipple disease confined to the central nervous system, white matter lesions initially appeared as areas of very low signal intensity on T1-weighted MR images and as areas of hyperintensity on proton density-weighted and T2-weighted images, and showed slight peripheral enhancement on delayed contrast-enhanced T1-weighted images. On MR studies obtained 3 and 6 months after antibiotic therapy, the lesions had decreased in size and no longer enhanced. They became progressively less hypointense on T1-weighted images and less hyperintense on T2-weighted images.

MRI of the spine in cobalamin deficiency: the value of examining both spinal cord and bone marrow.

We observed a case of pernicious anaemia in which MRI of the spine demonstrated both intrinsic lesions of the spinal cord and abnormal signal in the bone marrow. The latter resolved with replacement therapy. Only partial recovery of the cord lesions was observed.

Gout in the cervical spine: MR pattern mimicking diskovertebral infection.

We report the MR features of a surgically proved cervical spine involved with gouty tophi in a patient with a long history of hyperuricemia. Tophi appeared as sharply delineated areas of low signal intensity on T1 and T2 MR images and showed intense and homogeneous signal enhancement on post-contrast images.

Brain glucose utilization in band heterotopia: synaptic activity of "double cortex".

Regional brain glucose utilization was investigated with positron emission tomography and fluorodeoxyglucose in 2 patients with a seizure disorder associated with diffuse band heterotopia, a condition known as "double cortex." Although 1 patient was examined shortly after the onset of the first seizures, the other had a long history of intractable epilepsy before examination. Magnetic resonance imaging revealed a symmetric and generalized band of ectopic gray matter and an overlying normal-looking cortex, without focal abnormality. Metabolic studies yielded comparable results in both patients, with similar and even higher glucose uptake in the layer of gray matter heterotopia compared to the normal cortex. These data suggest the persistence of some synaptic activity in the heterotopic neurons, which seems unaffected by age or by the time-course of epilepsy.