Elodie Nerrant

Lack of confirmation of anti-inward rectifying potassium channel 4.1 antibodies as reliable markers of multiple sclerosis.

Background: auto-antibodies against the potassium channel inward rectifying potassium channel 4.1 (Kir4.1) have previously been identified in 46% of patients with multiple sclerosis (MS). Objectives: to confirm these findings. Methods: we evaluated the presence of anti-Kir4.1 antibodies by enzyme-linked immunosorbent assay (ELISA) and immunofluorescence in 268 MS patients, 46 patients with other neurological diseases (OND) and 45 healthy controls. Results: anti-Kir4.1 antibodies were found in 7.5% of MS patients, 4.3% of OND patients and 4.4% of healthy controls. Immunofluorescence analysis did not identify any specific staining. Conclusions: we confirmed the presence of anti-Kir4.1 antibodies in MS patients, but at a much lower prevalence than previously reported.

Hemolytic uremic syndrome: an unusual complication of interferon-β treatment in a MS patient

Interferon-b therapy is one of the most frequently used treatments in multiple sclerosis (MS), and is considered as safe. We report a patient with hemolytic uremic syndrome (HUS) related to recent prescription of interferon-b. A 38-year-old woman, without medical history, had a diagnosis of relapsing–remitting (RR) MS [1] (Fig. 1a). Subcutaneous interferon-b-1a (22 lg thrice weekly) was started THREE months later. Biological parameters including renal function were normal before interferon-b1a prescription. Seven months later, she presented asthenia, lower limb edema, and weight gain. She had no recent history of viral infection, transfusion, or additional drug intake. A few days later, she became oliguric. Physical examination showed high systolic and diastolic blood pressure (190/110 mmHg) and anasarca. Neurological examination was normal (EDSS score: 0). Biological parameters were the following: serum creatinine level 1,221 lmol/l (\90), urea level 31.8 mmol/l (\9), hemoglobin 4.6 g/dl (12–15). Initial platelet count was normal, followed by thrombocytopenia at 116,000/mm (250,000– 400,000). Occurrence of high LDH levels, immeasurable haptoglobin, and presence of 4 % of schistocytes, led to a diagnosis of hemolytic anemia. All autoimmune antibodies, including Coomb’s test, complement C3 and C4 levels, bacterial infection research, were normal or negative. The ADAMTS 13 activity was normal, without any ADAMTS 13 antibody and H or I factors deficit. Daily ultrafiltration and oral antihypertensive drugs were started, followed by plasma exchange and corticosteroid therapy (1 mg/kg/day). Renal biopsy confirmed acute thrombotic microangiopathy (TMA) (Fig. 1b). Absence of other causes and temporal relation between interferon-b and HUS led to a diagnosis of interferon-binduced HUS. Interferon-b-1a was withdrawn. The evolution was characterized by: (1) Three months of corticosteroids, hemodialysis, plasmapheresis, (2) occurrence of a typical posterior reversible encephalopathy syndrome (PRES) (Fig. 1c, d), secondary to the blood pressure imbalance, (3) definitive hemodialysis because of persistent anuria, (4) normalization of all hematological parameters. HUS is a type of thrombotic microangiopathy (TMA), as well as thrombotic thrombocytopenic purpura (TTP) [1–4]. HUS diagnosis is based on occurrence of mechanical hemolytic anemia, thrombocytopenia, and renal failure. High levels of LDH and reticulocytes, low haptoglobin levels, presence of schistocytes, and negative Coombs test, define the mechanical origin. Most cases are secondary to infection with Escherichia coli serotypes O157:H7, O111:H8, O103:H2, O123, O26, or other bacteria, such as E. Nerrant M. Charif P. Labauge (&) Department of Neurology, Montpellier University Hospital, 34295 Montpellier, France e-mail: labauge@yahoo.fr

Use of quantitative susceptibility mapping (QSM) in progressive multifocal leukoencephalopathy

BACKGROUND Progressive multifocal leukoencephalopathy (PML) is an opportunistic demyelinating encephalopathy related to JC virus. Its characteristics on conventional brain MRI are well known and are important for the diagnosis. OBJECTIVE To analyze SWI hypointensities recently described in U-fibers and cortex adjacent to the white matter lesions of PML. METHODS Prospective study including four patients with an history of definite diagnosis of PML. Clinical data were collected retrospectively. Brain MRI exams were done on a 3T magnet, including FLAIR, T2 GRE sequences and SWI. RESULTS Four males were included (mean age: 47 years, mean PML duration: 24.2 months). Immunosuppression was related to AIDS (n=2), natalizumab for multiple sclerosis (n=1), B-cell lymphoma treated by chemotherapeutic agents and rituximab (n=1). All patients had SWI hypointensities in cortex and/or U-fibers adjacent to the white matter lesions. QSM always suggested a paramagnetic effect. CONCLUSION SWI and T2 GRE hypointensities in cortex and U-fibers adjacent to the white matter lesions seem highly prevalent in PML, irrespective of the delay between PML onset and the MRI. QSM data suggest a paramagnetic effect.

Extensive cerebral white matter involvement in a patient with NMO spectrum disorder

Abnormal brain MRI has been described in up to 60% of patients with NMO patients. However, white matter T2 hyperintensities have been rarely observed. We report the case of a 49-year-old woman with long-lasting neuromyelitis optica (NMO) spectrum disorder and diffuse cerebral white matter T2-weighted hyperintensities. Our case suggests that some NMO patients can progressively develop l extensive cerebral involvement.

Teaching NeuroImages: Extensive vasogenic edema in Bickerstaff brainstem encephalitis

Ten days after respiratory infection, a 32-year-old woman presented with headaches, ataxia, and diplopia without encephalopathy. Brain MRI revealed extensive white matter, brainstem, and cerebellar vasogenic edema, without gadolinium enhancement, partially regressive during follow-up (figure, A). CSF revealed transient elevated protein level (1.01 g/L) and hypercellularity (123 neutrophils/mm3). Negative anti-GQ1b but positive anti-GD1a immunoglobulin G led to the diagnosis of Bickerstaff brainstem encephalitis (BBE). Symptoms resolved within 10 days without treatment. 18FDG-PET showed bilateral temporo-parieto-occipital and cerebellar hypometabolism (figure, B). Neurologists should be aware that diffuse brain hypometabolism or vasogenic edema can be associated with BBE.1,2

Deep brain stimulation treated dystonia-trajectory via status dystonicus: From Dystonia to Status Dystonicus

Background: Status dystonicus (SD) is a life‐threatening condition.

Early recurrence of migrainous infarction

A 47-year-old man with a history of migraine with visual aura, without other cardiovascular risk factors, presented with 3 migraine attacks with visual aura during a 11-day period (similar to previous attacks except for longer lasting visual aura, i.e. 85 min) was successfully treated by oral paracetamol. The last episode was followed by persisting visual disturbance. Three days later, brain MRI showed acute left-sided occipital (symptomatic) and subacute cerebellar (asymptomatic) infarctions (Fig. 1). Complete work-up in search of large-artery atherosclerosis, cardioembolism, coagulopathy, angiitis was normal. A diagnosis of recurrent migrainous infarction (MI) was made. MIs are predominantly located in the posterior circulation territory, mainly in the occipital lobes [1, 2]. Cerebellar involvement has been described in 21 % of MI cases [1]. The exact pathophysiological mechanisms of MI are unclear. In cortical spreading depression, neuronal activation is followed by hypoperfusion. In rare cases, it seems that hypoperfusion is severe enough to cause ischemia leading to infarction. Another hypothesis is that cerebral vasospasms, described to be associated with MI in some reported cases, may lead to brain ischemia [3]. Aura symptoms typically last longer ([ 60 min) in migraine with associated infarction. Early recurrence (based on the imaging characteristics of different brain infarctions on MRI, indicating infarctions at different age) of MI like in our case, however, is uncommon.