3 Tesla serial magnetic resonance imaging of human African trypanosomiasis (Trypanosoma brucei gambiense) and review of the literature.

We report serial magnetic resonance imaging (MRI) findings and follow-up in a case of human African trypanosomiasis (HAT) presenting with limited lesions followed by early and complete resolution. We searched the literature for documented cases and reviewed MRI findings before treatment. A 30-year-old Lebanese man, who had lived in Gabon for six years, presented with a two-year history of rash, anorexia, weight loss, arthralgia, paresthesia, and hypersomnia. Previously, the patient had received corticosteroid therapy for unconfirmed ANCA-associated vasculitis. Physical examination revealed a painless chancre on the left arm located at the site of an old insect bite, enlarged cervical, axillar and inguinal lymph nodes, hepatosplenomegaly and impaired concentration. Blood analysis showed an elevated protein level (90g/L) with hypoalbuminemia (24.2g/L) and elevated IgM (26.4g/L). Bone marrow aspirate and biopsy failed to detect any parasite. Polymerase chain reaction tests on blood and cerebrospinal fluid were positive for Trypanosoma. Serology tests confirmed the diagnosis of HAT due to Trypanosoma brucei gambiense infection. 3T MRI showed lesions in the hypothalamus and basal ganglia, the internal capsule, and the mesencephalon bilaterally. Follow-up MRI showed interval progression of the abnormalities. Treatment with melarsoprol was followed by clinical improvement with regression of the lesions on the three-month MRI, then total resolution at the 10-month follow-up. This case highlights a pattern of mild MRI lesions in T. brucei gambiense HAT with a total and rapid resolution under treatment. The literature review (16 HAT cases with sufficient radiological data, included ours) revealed an MRI pattern of brain lesion distribution that could be helpful for diagnosis and orienting biological tests.

Hemorrhagic presentation of frontal partially calcified pilocytic astrocytoma in an 18-year-old woman: A case report and literature review as "clinical case".

We report an unusual case of a frontal partially calcified pilocytic astrocytoma (PA) (WHO grade 1) in an 18-year-old woman who presented with acute, spontaneous intracerebral hemorrhage. Histopathology revealed the PA was mixed with psammoma bodies and areas of vascular proliferation responsible for a hypervascular pattern. The patient underwent a total gross resection. MRI showed no residual tumor at the 18-month follow-up and her neurological deficits improved after rehabilitation. Only 20 cases, including ours, of hemorrhagic presentation of PA in adults have been reported to date with enough radiological data. Furthermore, hemorrhagic presentation of a calcified PA is extremely rare. To date only two other cases of calcified PA with hemorrhagic presentation have been reported, one in an adult and one in an infant as described by Shibao et al. (2012) and Kapoor et al. (2015) respectively. Endothelial proliferation may be the main cause of bleeding in these lesions. In our case, a hypervascular pattern was exhibited by histopathological findings. A diagnosis of PA should be considered, especially when calcifications are present within a hemorrhagic tumor lesion.

Late onset hereditary episodic ataxia.

OBJECTIVE: Episodic ataxias (EA) are hereditary paroxysmal neurological diseases with considerable clinical and genetic heterogeneity. So far seven loci have been reported and four different genes have been identified. Analysis of additional sporadic or familial cases is needed to better delineate the clinical and genetic spectrum of EA. METHODS: A two generation French family with late onset episodic ataxia was examined. All consenting family members had a brain MRI with volumetric analysis of the cerebellum. Haplotype analysis was performed for the EA2 locus (19p13), the EA5 locus (2q22), the EA6 locus (5p13) and the EA7 locus (19q13). Mutation screening was performed for all exons of CACNA1A (EA2), EAAT1 (EA6) and the coding sequence of KCNA1 (EA1). RESULTS: Four family members had episodic ataxia with onset between 48 and 56 years of age but with heterogeneity in the severity and duration of symptoms. The two most severely affected had daily attacks of EA with a slowly progressive and disabling permanent cerebellar ataxia and a poor response to acetazolamide. Brain MRI showed in three affected members a decrease in the ratio of cerebellar volume:total intracranial volume, indicating cerebellar atrophy. No deleterious mutation was found in CACNA1A, SCA6, EAAT1 or KCNA1. In addition, the EA5 locus was excluded. CONCLUSIONS: A new phenotype of episodic ataxia has been described, characterised clinically by a late onset and progressive permanent cerebellar signs, and genetically by exclusion of the genes so far identified in EA.

MR imaging features of isolated cortical vein thrombosis: diagnosis and follow-up.

BACKGROUND AND PURPOSE: To our knowledge, very few MR imaging data have been reported in isolated cortical venous thrombosis (ICoVT). The purpose of this study was to describe MR imaging features, including T2*gradient-echo (GE) sequence, in presumed ICoVT. MATERIALS AND METHODS: MR imaging examinations were performed in 8 patients with ICoVT (MR venography was performed in all patients and digital substraction angiography in 4) at the time of diagnosis and during the follow-up at 15 days (4 patients) and at 3 (8 patients), 6 (6 patients), 12 (3 patients), and 18 months (1 patient). We assessed the presence of a magnetic susceptibility effect (MSE) on T2*GE imaging at each site of cerebral venous thrombosis and the presence or absence of a normal flow void and iso-, hypo-, or hyperintense signal intensity on T1, T2, diffusion-weighted imaging (DWI), and fluid-attenuated inversion recovery (FLAIR) images. Parenchymal signal-intensity changes were also assessed on the same sequences. RESULTS: MSE was detected on T2*GE imaging at the site of a cortical vein in all subjects at the first MR imaging examination. The occluded vein appeared as hyperintense in 3 patients, iso- to slightly hyperintense in 1 on T1, hypointense in 6 on FLAIR images, and as signal-intensity loss on DWI in 3. At follow-up, persisting signal-intensity abnormalities on T2*GE imaging were detected at the venous sites in all patients, whereas signal-intensity changes on T1- and T2-weighted images were no longer present. Parenchymal hyperintensities on FLAIR and DWI (increased apparent diffusion coefficient [ADC]) were observed in close vicinity to the thrombosis in 6/8 patients. Petechial hemorrhages (n = 3) or hematoma (n = 2) was present on T2*GE imaging in 5/8 patients. During the follow-up, all cerebral tissue signal-intensity changes on T1, T2, and FLAIR images decreased both in volume and intensity. ADC values normalized within the tissue after 3 months in all patients. CONCLUSIONS: On T2*GE imaging, MSE of hemoglobin products within the thrombus was observed both at the early and late phases of ICoVT and appears to be of high diagnostic value compared with the other signal intensity changes detected on standard MR imaging.

Clinical and brain MRI follow-up study of a family with COL4A1 mutation.

OBJECTIVE: To better delineate the clinical spectrum and the natural history of COL4A1 mutations, a newly defined genetic cause of small vessel disease including the brain and retina. METHODS: Clinical and brain MRI follow-up study of a family with COL4A1 mutation. RESULTS: During a 7-year period, two affected members died from intracranial hemorrhage. Four other members had a COL4A1 mutation (age ranges 25 to 74 years). None reported stroke or retinal hemorrhage or hematuria and none had dementia according to Diagnostic and Statistical Manual of Mental Disorders-IV criteria. Follow-up brain MRI showed grade 3 diffuse leukoencephalopathy in three out of four patients. All had dilated perivascular spaces and three out of four had silent microbleeds mainly in the deep white matter. MRI signal abnormalities did not change in severity, number, or location between baseline and follow-up imaging. CONCLUSIONS: COL4A1 mutation carriers have great diversity in the clinical expression of the disease within the same family. Some affected family members may remain asymptomatic during several years of follow-up and have no evidence of progression of vascular changes on brain MRI.

[CT and MRI features of arachnoid cyst of the petrous apex: report of 3 cases]. / Aspects TDM et IRM du kyste arachnoïdien de l'apex pétreux.

PURPOSE: To describe the CT and MRI features of 3 cases of arachnoid cyst of the petrous apex. PATIENTS AND METHODS: Three patients with isolated trigeminal neuralgia, trigeminal hypoesthesia, and sinusitis. Axial and coronal CT images were obtained. T1W, FSE T2W, FLAIR, T2*W and diffusion-weighted MR sequences were obtained. RESULTS: In all cases, both CT and MRI showed expansile lesions eroding the petrous apex. Lesions were hypodense on CT and isointense to CSF on MRI, without contrast enhancement. In one case, the lesion was contiguous with Meckel's cave with temporal fossa and sphenoid sinus extension. CT and MR imaging features are useful to distinguish arachnoid cysts of the petrous apex from other benign lesions of the petrous apex. CONCLUSION: CT and MRI imaging features allow diagnosis of arachnoid cyst of the petrous apex because its imaging features, especially on DWI, are different from other cystic lesions of the petrous apex, namely cholesteatoma. It should be considered in patients with trigeminal involvement, especially trigeminal neuralgia.

Orgasmic headache and middle cerebral artery dissection.

A patient with a 20 year history of primary orgasmic headache is described who, after suffering an unusually severe episode of orgasmic headache was found to have a middle cerebral artery dissection. This unusual association of primary and secondary orgasmic headache emphasises the need for a thorough diagnostic examination when the orgasmic headache differs from that of previous episodes or is associated with neurological symptoms.

Early epidural blood patch in spontaneous intracranial hypotension.

Thirty patients with a typical orthostatic headache were treated by early lumbar epidural blood patch (EBP) without previously performing lumbar puncture or identifying a CSF leak and with or without typical MRI changes. A complete cure was obtained in 77% of patients after one (57%) or two (20%) EBPs. Spontaneous intracranial hypotension with typical orthostatic headache can be diagnosed without lumbar puncture and can be cured by early EBP in a majority of patients.

Facial "glomangiomas": large facial venous malformations with glomus cells.

BACKGROUND: "Glomangiomas" are benign cutaneous vascular lesions consisting of convoluted, abnormally formed venous channels lined by cuboidal and oval epithelioid, alpha-actin-positive, glomus cells. Three different clinical variants of glomangioma have been recognized: solitary, multiple, and nodular, or plaquelike. Inheritable forms are common. OBJECTIVE: We describe in 7 patients (2 of them having a familial glomangiomatosis) the rare facial location of glomangiomas to differentiate this type from common facial venous malformation (VM). METHODS: We analyzed clinical data (photographs), course, investigations (computed tomographic scans in 4 patients, magnetic resonance imaging in 6, arteriography in 2, direct puncture phlebography in 4, and pathologic examinations in all 7), and outcome with treatment. RESULTS: Lesions were soft, composed of multiple nodules, confluent and plaquelike, deep blue or blue-to-purple, sometimes sagging, one-sided in a cheek, extending to the lips in 5 patients, to the chin in 4, and to the lower eyelid in 4. They were poorly compressible, a finding different from common facial VMs. In a young man extensive back involvement was associated. Among radiologic investigations, only magnetic resonance imaging after gadolinium enhancement offered some differential features with common VMs. However, histopathologic examination clarified the differential diagnosis: although the large tortuous venous channels were reminiscent of capillary-venous malformation, in many vessels the walls contained one or several rows of glomus cells. CONCLUSION: Multiple plaquelike facial "glomangiomas" mimic a common venous malformation because of their blue hue. However, with experience, one can clinically recognize them, and their pathologic aspect is distinctive. Management should differ slightly from that for common facial VM because sclerotherapy has proven to be less effective. Therefore surgical treatment is the only helpful therapeutic option.

Spinal subdural haematoma: a study of three cases.

AIMS: To define the clinical, computed tomography (CT) and magnetic resonance imaging (MRI) features and the role of MRI in the follow-up of spinal subdural haematoma (SSH), and to compare these findings with those of spinal epidural haematomas (SEH). METHODS: We report three cases of SSH (two women, one male, age: 50-74 years). Two patients were on anticoagulant therapy; in the other case the SSH was spontaneous. All the patients were examined 1-3 days after the onset of the symptoms. All of them had CT, two had MRI and one had angiography. Two patients underwent surgery. RESULTS: The haematoma was located in the thoracolumbar region (two) and in the thoracic region (one), extending from five (two) to 11 vertebral body levels (one). The haematomas were posteriorly located with lateral extension. The transverse shape differed with the level: biconvex, biloculated or circumferential. The haematomas were hyperdense on CT. On MRI, SSH yielded high signal on both T1 and T2. The integrity of the posterior fat pads, which was well shown on CT and MRI, and the visualization of the dura mater demonstrated the intradural location of these collections, making them easily distinguishable from spinal epidural haematoma. MRI provides better evaluation of the longitudinal extent. Our results are compared with those reported in the literature. CONCLUSION: MRI is superior to CT for diagnosis and follow-up of SSH. Our findings and those reported in the literature show that the MR features of SSH are quite specific and allow differentiation from SEH.

[Study of the middle ear. Value of multiplanar reconstructions in spiral tomodensitometry]. / Etude de l'oreille moyenne. Intérêt des reconstructions multiplanaires en tomodensitométrie spiralée.

We point out the interest of computed tomographic reconstructions from spiral acquisition--particularly sagittal reconstructions--in the study of middle ear anatomy and adjacent structures: the facial canal and the chorda tympani. The reference reconstructions are axial and coronal reconstructions. So, we demonstrate the superiority of sagittal reconstructions for the visualization of the lateral process of the malleus, the body and long process of the incus, the third portion of the facial canal, and the chorda tympani. For the other structures of the middle ear and the other parts of the facial canal, these sagittal reconstructions are complementary. Besides, the best type of reconstruction to visualize the stapes and the vestibular window is the axial plane parallel to the stapes axis.

Cranial epidural tuberculoma. A case report.

A 33-year-old patient with cranial epidural tuberculoma without history of tuberculosis is described. CT and MR imaging showed a lesion located on both sides of a right frontotemporal bone destruction with epidural extent. Except for a small necrotic core, the lesion enhanced intensely after contrast medium administration. Osteitis and subgaleal abscess were associated. The displaced dura mater delineated the epidural tuberculoma. Diagnosis was verified by histology and identification of Mycobacterium tuberculosis. After removal of the tuberculoma and combination therapy, there was a complete regression of abnormalities. Differential diagnoses are dural tuberculoma, focal tuberculous pachymeningitis and tuberculous epidural empyema.

[Sturge-Weber syndrome. The current neuroradiologic data]. / Syndrome de Sturge-Weber. Données actuelles de l'imagerie neuroradiologique.

Sturge-Weber syndrome (SWS) is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain (PWS) involving the V1 facial trigeminal skin area, alone or in combination with V2 and V3PWS, seizures and ocular abnormalities (glaucoma and choroidal angioma). Radiologically, a leptomeningeal (pial) capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Developmental venous anomalies (DVA) of the brain are sometimes associated. MR with gadolinium enhancement is the optimal neuro-diagnostic imaging technique for the screening of infants with an at-risk V1PWS, as well as for the follow-up of patients with evidence SWS. Accelerated myelination in the involved hemisphere may be an early diagnostic feature before 6 months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. A neonatal neuro-imaging work-up, using CT or MRI, may not demonstrate the pial anomaly and should be repeated after 6 to 12 months in an at-risk infant with V1PWS.

Deep cerebral venous thrombosis: imaging in eight cases.

Deep cerebral venous thrombosis (DCVT) is a rare, potentially fatal disease. We carried out a retrospective analysis of eight patients presenting with DCVT, using CT and MRI data. Digital subtraction angiography (DSA), MR angiography (MRA) (2D phase-contrast), and angiography were performed in four, four and two patients, respectively. Direct (venous abnormalities) and indirect (parenchymatous involvement) signs of thrombosis were assessed. Follow-up MRI and MRA were performed on two patients. CT was initially normal in two patients, demonstrated a "cord sign" in deep veins in five and deep venous infarcts in three. When performed within a week of admission (seven patients), MRI showed thrombosis as into intense or high signal in deep veins on sagittal T1-weighted spin-echo images in all patients, and on axial T2-weighted spin-echo images in two. Deep venous infarcts were found in five patients. Direct or indirect signs of sagittal or lateral sinus thrombosis were present on CT in two patients and on MRI in five. CT angiography, MRA and DSA were concordant with MRI findings. CT venography showed persistent flow in thrombosed veins. MRI follow-up demonstrated progressive deep venous recanalisation.

Cerebral amyloid angiopathy (CAA) with presentation as a brain inflammatory pseudo-tumour.

Cerebral amyloid angiopathy (CAA) is frequent but often asymptomatic. It can induce lobar haemorrhage, rapidly progressive dementia or recurrent transient neurological symptoms, other presentations being less frequent. We report 3 patients in their sixties presenting with a space occupying lesion which was the first manifestation of CAA. They were operated with a diagnosis of cerebral tumour. In all three cases, macroscopy was similar, the lesions were superficial in the cerebral cortex and the preoperative diagnoses were glioblastoma, meningioma and cavernoma. Histologically, the lesions consisted of a large inflammatory granuloma with numerous lipophages and siderophages surrounding capillaries with prominent endothelial cells. Vessels in the near cortex and meninges and within the granuloma harboured heavy amyloid deposits immunolabelled by anti-P component, anti-protein beta A4 with a A40 predominance and anti-apolipoprotein E. Adjacent cerebral cortex showed reactive gliosis and rare senile plaques. Amyloidosis is rarely considered among diagnoses of space occupying lesions. In our three cases, CT scan and MRI changes were related to the presence of an inflammatory granuloma around foci of haemorrhage and amyloid laden vessels.

[Tuberculosis of the central nervous system. MRI features and clinical course in 12 cases]. / Tuberculose du système nerveux central. Aspects IRM et évolution à propos de 12 cas.

OBJECTIVE: The aim of this study was to characterize the MR findings of various intracranial lesions in the central nervous system (CNS) tuberculosis. MATERIALS AND METHODS: The MRI findings (T1, T2 and T1 sequence after contrast) of 12 proved cases (10 males, 2 females, 24 to 64 years old, average: 35) of CNS tuberculosis were reviewed; 4 patients were seropositive for HIV. RESULTS: Several types of lesions were identified: tuberculomas called miliary lesions if they were smaller than 2 mm in diameter (7 cases), in 1 case the tuberculoma was revealed by two large lesions and bi-hemispheric localisations, leptomeningitis (5 cases), infarction (4 cases), abscesses (3 cases with solitary lesions in 2/3 cases), hydrocephalus (3 cases), pachymeningitis (2 cases). A tuberculomas-leptomeningitis association was found in 4 patients. The pachymeningitis form had an unusual aspect in one case. Patients with leptomeningitis showed thick meningeal contrast enhancement involving all basal cisterns, expanding to the sylvian fissures level, and causing narrowing of the sylvian arteries. Massive infarctions resulted from arterial englobement or embols. In three out of five patients, leptomeningitis was the initial presentation. In seropositive patients, tuberculosis was severe with high mortality (3/4 patients), and associated with other multiple lesions. CONCLUSION: Central nervous system tuberculosis has different appearances, mostly tuberculomas and leptomeningitis. MR with contrast is necessary for follow-up during treatment.