Silent triggers and symmetric peduncles - a rare presentation of adult-onset acute disseminated encephalomyelitis: A case report

Central nervous system (CNS) involvement has been observed in 14-80% of patients with systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI) is an appropriate method for evaluating CNS involvement in these patients. Clinical manifestations and MRI findings of CNS lupus should be differentiated from other mimicking diseases such as multiple sclerosis (MS). The aim of this study was to evaluate the prevalence and extent of brain and cervical cord MRI lesions of lupus patients. The relationship between neurological signs and symptoms and MRI findings were evaluated as well. Fifty SLE patients who had been referred to the rheumatology clinic of our hospital within 2009 were included in a cross sectional study. All patients fulfilled the revised 1981 American College of Rheumatology (ACR) criteria for SLE. We evaluated the neurological signs and symptoms and brain and cervical MRI findings in these patients. Forty-one patients (82%) were female and nine (18%) were male. The mean age was 30.1 ± 9.3 years. Twenty eight (56%) patients had an abnormal brain MRI. No one showed any abnormality in the cervical MRI. The lesions in 20 patients were similar to demyelinative plaques. Seventeen patients with abnormal brain MRI were neurologically asymptomatic. There was only a significant relationship between neurological motor manifestations and brain MRI abnormal findings. Unlike the brain, cervical MRI abnormality and especially asymptomatic cord involvement in MRI is quite rare in SLE patients. This finding may be helpful to differentiate SLE from other CNS disorders such as MS.

Clinically differentiating multiple system atrophy cerebellar (MSA-C) phenotype and spinocerebellar ataxias (SCAs) is challenging especially in the early stage. We assessed diagnostic value of brain magnetic resonance imaging (MRI) in differentiating MSA-C and SCAs based at different disease stages (<3, 3–7, and >7 years of disease duration). Overall, 186 patients with probable MSA-C and 117 with genetically confirmed SCAs were included. Hot cross bun (HCB) signs and middle cerebellar peduncle (MCP) hyperintensities were exclusively prevalent in MSA-C compared to SCAs at <3 years (HCB, 44.6% versus 0.9%; MCP hyperintensities, 38.3% versus 0.9%, respectively). Sensitivity, specificity, and positive predictive value (PPV) for HCB signs to differentiate MSA-C from SCAs were 45%, 99%, and 99% and those for MCP hyperintensities were 68%, 99%, and 99%, respectively; considering both HCB signs and MCP hyperintensities, specificity and PPV were 100%. However, the differential value of MRI signs decreased over time. MCP widths were smaller and showed more significant decrease in MSA-C than in SCAs. In conclusion, pontine and MCP changes were exclusively prominent in early stage MSA-C rather than in SCAs. Therefore, we should consider disease duration when interpreting pontine and MCP changes in brain MRIs, which will help better differentiate MSA-C and SCAs.

A 43-year-old woman was admitted in our department for progressive ataxia, headache and altered general status lasting for 3 weeks. Brain magnetic resonance imaging (MRI) showed multifocal lesions in hypersignal T2 and with homogeneous gadolinium enhancement in T1 sequences (Figure 1). Extensive cerebrospinal fluid (CSF) examination with isoelectric focusing, search for malignant or lymphomatous cells, microbacterial analysis, including China ink and mycobacterium cultures and polymerase chain reaction (PCR), trophyrema whippeli PCR was normal. Thorax and abdomen computerized tomography (CT) scan, bone scintigraphy, whole body positron emission tomography (PET) fluorodesoxyglucose (FDG) scan, bones X-rays and bone marrow biopsy did not show any abnormality. Figure 1. Axial and sagittal brain and spine MRI views showing multifocal lesions involving the corpus callosum, right middle cerebellar peduncle, right periventricular white matter, cervical and …

57-Year-Old Woman With Fever and Confusion

Central Nervous System (CNS) involvement of Systemic Lupus Erythematosus (SLE) includes a broad range of neuropsychiatric syndromes. Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating CNS disorder characterized by encephalopathy and multifocal lesions predominantly involving the white matter on brain magnetic resonance imaging. ADEM associated with SLE has been only rarely reported. We report an unusual case of a 17-year-old girl who developed ADEM after enteroviral infection as the first manifestation of SLE. The authors emphasize that the patient's illness was preceded by enteroviral infection and that ADEM occurred before any other symptoms of SLE, which makes this case unique.

To develop a decision tree based on standard magnetic resonance imaging (MRI) and diffusion tensor imaging to differentiate multiple system atrophy (MSA) from Parkinson's disease (PD). 3-T brain MRI and DTI (diffusion tensor imaging) were performed on 26 PD and 13 MSA patients. Regions of interest (ROIs) were the putamen, substantia nigra, pons, middle cerebellar peduncles (MCP) and cerebellum. Linear, volumetry and DTI (fractional anisotropy and mean diffusivity) were measured. A three-node decision tree was formulated, with design goals being 100 % specificity at node 1, 100 % sensitivity at node 2 and highest combined sensitivity and specificity at node 3. Nine parameters (mean width, fractional anisotropy (FA) and mean diffusivity (MD) of MCP; anteroposterior diameter of pons; cerebellar FA and volume; pons and mean putamen volume; mean FA substantia nigra compacta-rostral) showed statistically significant (P < 0.05) differences between MSA and PD with mean MCP width, anteroposterior diameter of pons and mean FA MCP chosen for the decision tree. Threshold values were 14.6 mm, 21.8 mm and 0.55, respectively. Overall performance of the decision tree was 92 % sensitivity, 96 % specificity, 92 % PPV and 96 % NPV. Twelve out of 13 MSA patients were accurately classified. Formation of the decision tree using these parameters was both descriptive and predictive in differentiating between MSA and PD. • Parkinson's disease and multiple system atrophy can be distinguished on MR imaging. • Combined conventional MRI and diffusion tensor imaging improves the accuracy of diagnosis. • A decision tree is descriptive and predictive in differentiating between clinical entities. • A decision tree can reliably differentiate Parkinson's disease from multiple system atrophy.

BackgroundWallerian degeneration (WD) can occur in different projecting systems, such as corticospinal tract, dentate-rubro-olivary pathway, and corticopontocerebellar tract. However, the co-occurrence of hypertrophic olivary degeneration (HOD) and middle cerebellar peduncles (MCPs) degeneration secondary to unilateral pontine infarction in a single patient is extremely rare.Case presentationA 71-year-old man presented with acute onset of dizzness, slurred speech, and right-sided weakness. On the next day, his previous neurologic deficits deteriorated. Brain magnetic resonance imaging (MRI) revealed acute ischemic stroke of the left pons. After treatment with thrombolysis, antiplatelets, and rehabilitation training, his speaking and motor function improved moderately. At the 3-month follow-up, the MRI showed hyperintensity in the left medulla oblongata and bilateral MCPs on T2-weighted and FLAIR images, suggesting HOD as well as MCPs degeneration.ConclusionsIt is of great importance for us to know the anatomic knowledge of dentate-rubro-olivary and corticopontocerebellar pathways.

To the Editor: Varicella-zoster virus (VZV) infection can lead to zoster or cranial nerve palsy such as Ramsay Hunt syndrome (RHS), or complications of central nervous system (CNS) such as myelitis, cerebellitis, encephalitis, and stroke syndrome.[1,2] However, the coexistence of RHS and VZV brainstem encephalitis is extremely rare to our knowledge. We, therefore, report a patient who presented with RHS and multiply cranial nerve palsies. A 58-year-old immunocompetent man felt febrile and severe otalgia with a frequent dry cough. One week later, he gradually developed left facial paralysis, left ear hearing loss, and vertigo. After another 3 days, dysarthria, swallowing difficulty, and intractable hiccup presented sequentially. On the next day, he complained of diplopia with extraocular movement limitation and was admitted to the Second Affiliated Hospital of Nanchang University. On admission, his vital signs were stable. Neurological examinations showed the following: Left eye immobilization with right eye adduction limitation, left facial weakness and hypesthesia, left sensorineural hearing loss, paralysis of the left soft palate, and slurred speech. There were slightly decreased muscle power and hyperreactive tendon reflexes with a positive Barbinski sign contralaterally. Coordinate movement of four limbs was impaired, and the Romberg test was positive. Carefully, herpetic vesicles, erythematous ulcerative, and crusted scars were observed around the left external acoustic meatus. Brain magnetic resonance imaging (MRI), on admission [Figure 1], revealed a high signal intensity lesion involving basis pontis and medulla oblongata on both the T2-weighted image [Figure 1b] and fluid attenuation inversion recovery (FLAIR) [Figure 1d]. Magnetic resonance angiography showed normal findings. Moreover, contrast-enhanced MRI showed a spot-like enhancement in medulla oblongata as well as enhanced left facial nerve [Figure ​[Figure1e1e and ​and1f].1f]. On day 2 after admission, an electroencephalogram showed diffuse theta waves. Meanwhile, the cerebrospinal fluid (CSF) analysis showed an increased cell count (1360/mm3, 80% lymphocytes) and protein level (1.37 g/L). CSF cultures for bacteria, fungus, tuberculosis, and herpes simplex virus DNA were negative. This patient was immediately administrated with intravenous acyclovir (10 mg/kg every 8 h) and methylprednisolone (40 mg/d) after hospitalization. Three days after admission the patient deteriorated with paralysis of bilateral limbs and pulmonary inflammation. He was treated with empiric antibiotics. On day 7, an elevated serum IgM antibody titer to VZV on enzyme-linked immunosorbent assay (ELISA) and the presence of CSF VZV DNA amplified by polymerase chain reaction (PCR) confirmed VZV infection. On day 14, his vertigo, swallowing and speaking function improved moderately, but his hearing loss and facial palsy remained unaltered. One month later, follow-up brain MRI demonstrated that the extent of the lesion had decreased [Figure ​[Figure1g1g–1I] and PCR for VZV DNA in CSF became negative. Clinically, he could walk alone and hear high tone voice in his left ear. Figure 1 On admission, brain magnetic resonance imaging (MRI) shows the lesion in the basis pontis and medulla oblongata: T1-weighted hypointense (a), T2-weighted (b) and fluid attenuation inversion recovery (FLAIR) hyperintense (d), and slightly diffusion-weighted ... VZV is a member of the family Herpesviridae with the ability to establish latency in dorsal root-, autonomic- and cranial ganglia. After reactivation, VZV causes herpes zoster in most cases. VZV might also infect CNS causing various neurological manifestations.[3,4] With the introduction of using PCR for detection of virus DNA in CSF, VZV has been reported to be the second most common viruses of encephalitis recently.[5] Based on herpes zoster with facial nerve palsy, our patient fulfills the criteria for RHS. Moreover, he can also be diagnosed with VZV brainstem encephalitis according to CSF evidence of VZV and pontobulbar involvement on brain MRI. However, reports about RHS accompanied by VZV encephalitis have been rarely documented in the literature. The reason for the low incidence of RHS complicated by VZV encephalitis is not well understood. Clinically, our case was initially diagnosed with RHS. Subsequently, he presented with multiple cranial nerve palsies (from III to X cranial nerves) and central spastic palsy (bilateral pyramidal tracts involvement), which may suggest a brainstem insult. Furthermore, transverse hyperintensities in central pons on T2-FLAIR obviously showed the lesions. Meanwhile, contrast-enhanced MRI showed enhanced left facial nerve. We speculate the possible mechanism of VZV spreading to CNS is the reactivated viruses, which establish latency in geniculate ganglia, upward thread porus acusticus internus along with facial canal, and eventually enter intracranially and firstly invade basis pontis. Meanwhile, VZV may also spread downwards along with general somatosensory fibers to the skin of external auditory canal resulting in herpes zoster formation. To the best of our knowledge, coexistence of RHS with VZV brainstem encephalitis is extremely rare. This case may widen our knowledge for the mechanism of VZV infection spreading into CNS. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Radiation-Induced Multiphasic Demyelination.

Conséquences familiales du diagnostic de FXTAS : le conseil génétique aux apparentés à risque

Clinical and radiological features of recurrent demyelination following acute disseminated encephalomyelitis (ADEM)

Central nervous system vasculitis from Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorder in children: A case report

Background and ObjectivesMyelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with an age-related phenotypic spectrum. At disease onset, there is considerable clinical overlap between MOGAD and other demyelinating conditions, and it remains difficult to identify MOGAD radiographically. This study aims to further describe neuroimaging findings in the brain and the spine at presentation and throughout relapses in children with MOGAD.MethodsWe present a retrospective cohort study including all children presenting to a single center between 2010 and 2020 with acute demyelination who were positive for serum MOG-IgG antibodies and negative for serum aquaporin-4 antibodies. For each patient, magnetic resonance imaging (MRI) scans of the brain and spine at presentation and on each relapse were reviewed and categorized in a blinded fashion by 2 pediatric neuroradiologists.ResultsSixteen patients met the inclusion criteria. Four had diffuse and bilateral fluid-attenuated inversion recovery signal in the white matter, but only on initial presentation. The area postrema was never affected. All 5 patients with optic neuritis had pre-chiasmatic (but not chiasmatic) involvement on presentation. The brachium pontis was involved in 3 patients on initial presentation, and in 8 patients at any time. Eleven patients demonstrated spinal cord involvement, and the cervical, thoracic, and lumbar regions were involved at similar frequencies.DiscussionThe radiographic features of MOGAD in children appear to reflect their presenting demyelinating syndromes. However, certain features, such as diffuse fluid-attenuated inversion recovery hyperintensities and expansile fluid-attenuated inversion recovery signal in the brachium pontis, may be more frequent in MOGAD compared with other demyelinating disorders.

A 44-year-old female, with a history of severe hypertension, presented with subacute onset of headache and visual deterioration. The general and neurological examination was unremarkable. The ophthalmologic examination showed signs of chronic hypertensive retinopathy. Blood pressure was 230/130 mm Hg. Initial brain computed tomography (CT) scan revealed the presence of hypodensity in the pons, cerebellum, and thalamus (Figure 1A and ​andB).B). On brain magnetic resonance imaging (MRI), the lesions were consistent with vasogenic edema involving the brain stem, cerebellum, thalamus, and basal ganglia (Figure 1C-​-J).J). The remaining investigation (hemogram, biochemical, echocardiogram, renal and suprarenal echography, and lumbar puncture) was unremarkable. The blood pressure was lowered to 150 to 140 mm Hg and the diastolic blood pressure to 110 to 100 mm Hg with amlodipine, Ramipril, and furosemide. After 2 weeks, she was completely asymptomatic. The repeated MRI of the brain revealed complete resolution of the previous lesions (Figure 2). Isolated involvement of the brain stem and cerebellum is an extremely rare central variant of posterior reversible encephalopathy syndrome (PRES).1 The combination of severe hypertension, clinical–radiological dissociation, and brain MRI with findings compatible with vasogenic edema is typical of the central variant of PRES.1,2 This triad should promptly guide the treatment toward blood pressure normalization and avoid unnecessary investigations. Figure 1. Initial brain computed tomography (CT) showing hypodensity in the pons, cerebellum, and thalamus (A and B), axial brain magnetic resonance imaging (MRI) showing hyperintensity in the pons, midbrain, middle cerebellar peduncles, and thalamus on fluid-attenuated ... Figure 2. Follow-up brain magnetic resonance imaging (MRI) showing complete resolution of the cerebral edema on fluid-attenuated inversion recovery (FLAIR; A-D) and coronal T2-weighted (E) and normal sign on DWI-weighted (F), ADC map (G), and T1-coronal T1-weighted ...

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare variant of CAA with autoimmune inflammation. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Brain magnetic resonance imaging (MRI) revealed an area of abnormal signal and mild parenchymal swelling in the right parietal lobe, indicating vasogenic edema. T2⁎-weighted gradient echo imaging revealed some subcortical microbleeds in the same lesion. Based on the proposed criteria for CAA-ri, she was diagnosed with probable CAA-ri. After 4 months, the spontaneous improvement was noted in the patient's clinical and radiological findings. This report presents a rare and atypical case of CAA-ri in which the diagnosis was established after the patient underwent neuroimaging for only mild neurological symptoms, and the patient's clinical and radiological findings displayed spontaneous improvement. Despite typical and striking MRI findings of CAA-ri, this patient only presented a minimal symptom; this dissociation could highlight the significance of not misinterpreting any new neurological symptoms. Thus, increased availability of MRI and growing awareness of CAA-ri might result in more incidentally diagnosed cases in the future. Furthermore, this case suggests that it would be better to strictly monitor the clinical-radiological findings of patients with probable CAA-ri who only present with minimal symptoms without the initiation of immunosuppressive therapy.