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Simona Mihaela Florea, Alice Faure, Hervé Brunel, Nadine Girard and Didier Scavarda

The embryological development of the central nervous system takes place during the neurulation process, which includes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD.

This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5–S1 level, with a thickened filum terminale.

The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale.

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Olivier Levrier, Philippe Métellus, Stephane Fuentes, Luis Manera, Henry Dufour, Anne Donnet, François Grisoli, Jean-Michel Bartoli and Nadine Girard

Object

The goal of this study was to evaluate the clinical and angiography results in 10 patients with transverse–sigmoid dural arteriovenous fistulas (DAVFs) treated using sinus angioplasty and dural sinus stent insertion.

Methods

Between 2001 and 2003, 10 consecutive patients (six men and four women, age range 54–79 years) who had presented with transverse and/or sigmoid sinus DAVFs with or without sinus thrombosis underwent self-expanding stent placement and balloon angioplasty. Eight fistulas involved the transverse sinus, three the sigmoid sinus, and one the torcular and occipital sinuses. According to the Djindjian-Merland grading system, there were two Type I, five Type IIa, one Type IIb, and two Type IV DAVFs. The mean clinical follow-up period was 21.1 months. At the last follow-up examination, seven patients were asymptomatic and three were dramatically improved. The mean angiography follow-up period was 7.5 months for the available population: four patients had complete DAVF occlusion, four had significant flow reduction, and two who experienced clinical improvement refused conventional angiography control studies. Delayed computerized tomography angiography scans were obtained to evaluate stent permeability in nine of the 10 patients. Stent permeability was demonstrated in eight of the nine patients with available control studies at a mean follow up of 20.8 months. There were two transient neurological deficits but no severe and permanent complications.

Conclusions

In this series, sinus stent insertion resulted in a cure or significant clinical improvement in all patients harboring a DAVF, with no severe or permanent complication. Stent placement for transverse and/or sigmoid sinus DAVFs is a promising technique whose viability should be confirmed in larger series with longer follow-up periods.

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Didier Scavarda, Tiago Cavalcante, Agnès Trébuchon, Anne Lépine, Nathalie Villeneuve, Nadine Girard, Aileen McGonigal, Mathieu Milh and Fabrice Bartolomei

OBJECTIVE

Hemispherotomy is currently the most frequently performed surgical option for refractory epilepsy associated with large perinatal or childhood ischemic events. Such an approach may lead to good seizure control, but it has inherent functional consequences linked to the disconnection of functional cortices. The authors report on 6 consecutive patients who presented with severe epilepsy associated with hemiplegia due to stroke and who benefitted from a new, stereoelectroencephalography-guided partial disconnection technique.

METHODS

The authors developed a new disconnection technique termed “tailored suprainsular partial hemispherotomy” (TSIPH). Disconnection always included premotor and motor cortex with variable anterior and posterior extent.

RESULTS

At a mean follow-up of 28 months, there were no deaths and no patient had hydrocephalus. Motor degradation was observed in all patients in the 2 weeks after surgery, but all patients completely recovered. The 6 patients were seizure free (Engel class IA) at the last follow-up. No neuropsychological aggravation was observed.

CONCLUSIONS

TSIPH appears to be a conservative alternative to classic hemispherotomy, leading to favorable outcome in this series.

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Constantin Tuleasca, Jean Régis, Elena Najdenovska, Tatiana Witjas, Nadine Girard, Thomas Bolton, Francois Delaire, Marion Vincent, Mohamed Faouzi, Jean-Philippe Thiran, Meritxell Bach Cuadra, Marc Levivier and Dimitri Van de Ville

OBJECTIVE

Essential tremor (ET) is the most common movement disorder. Drug-resistant ET can benefit from standard stereotactic deep brain stimulation or radiofrequency thalamotomy or, alternatively, minimally invasive techniques, including stereotactic radiosurgery (SRS) and high-intensity focused ultrasound, at the level of the ventral intermediate nucleus (Vim). The aim of the present study was to evaluate potential correlations between pretherapeutic interconnectivity (IC), as depicted on resting-state functional MRI (rs-fMRI), and MR signature volume at 1 year after Vim SRS for tremor, to be able to potentially identify hypo- and hyperresponders based only on pretherapeutic neuroimaging data.

METHODS

Seventeen consecutive patients with ET were included, who benefitted from left unilateral SRS thalamotomy (SRS-T) between September 2014 and August 2015. Standard tremor assessment and rs-fMRI were acquired pretherapeutically and 1 year after SRS-T. A healthy control group was also included (n = 12). Group-level independent component analysis (ICA; only n = 17 for pretherapeutic rs-fMRI) was applied. The mean MR signature volume was 0.125 ml (median 0.063 ml, range 0.002–0.600 ml). The authors correlated baseline IC with 1-year MR signatures within all networks. A 2-sample t-test at the level of each component was first performed in two groups: group 1 (n = 8, volume < 0.063 ml) and group 2 (n = 9, volume ≥ 0.063 ml). These groups did not statistically differ by age, duration of symptoms, baseline ADL score, ADL point decrease at 1 year, time to tremor arrest, or baseline tremor score on the treated hand (TSTH; p > 0.05). An ANOVA was then performed on each component, using individual subject-level maps and continuous values of 1-year MR signatures, correlated with pretherapeutic IC.

RESULTS

Using 2-sample t-tests, two networks were found to be statistically significant: network 3, including the brainstem, motor cerebellum, bilateral thalamus, and left supplementary motor area (SMA) (pFWE = 0.004, cluster size = 94), interconnected with the red nucleus (MNI −2, −22, −32); and network 9, including the brainstem, posterior insula, bilateral thalamus, and left SMA (pFWE = 0.002, cluster size = 106), interconnected with the left SMA (MNI 24, −28, 44). Higher pretherapeutic IC was associated with higher MR volumes, in a network including the anterior default-mode network and bilateral thalamus (ANOVA, pFWE = 0.004, cluster size = 73), interconnected with cerebellar lobule V (MNI −12, −70, −22). Moreover, in the same network, radiological hyporesponders presented with negative IC values.

CONCLUSIONS

These findings have clinical implications for predicting MR signature volumes after SRS-T. Here, using pretherapeutic MRI and data processing without prior hypothesis, the authors showed that pretherapeutic network interconnectivity strength predicts 1-year MR signature volumes following SRS-T.