To determine the safety and tolerability of home-based intravenous immunoglobulin (IVIG) (Gamunex) as maintenance treatment in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in Canada.

We enrolled ten subjects with CIDP who had previously received IVIG in the hospital setting to receive the comparable IVIG dose (1-2 g/kg/month) in the home for six months. The patients were evaluated in the clinic at three months and at six months to evaluate their clinical status as well as the safety and tolerability of IVIG.

All subjects tolerated home-based IVIG treatment as maintenance treatment of CIDP. There were no serious adverse events related to IVIG. Subjects did experience “anticipated” IVIG events post-infusion such as headache and fatigue, which were managed with analgesics and supportive counseling. One subject withdrew consent at end of study due to hospitalization. This event was not related to the IVIG. Another subject experienced a “flare” of CIDP symptoms near the end of the study, however, completed all visits as per protocol. All subjects expressed excellent satisfaction with the individualized therapy, and almost all (nine out of ten) patients preferred home-infusion to hospital-infusion.

Intravenous immunoglobulin can be delivered safely and is well tolerated outside the hospital setting in Canada in patients with chronic, stable neuromuscular conditions such as CIDP who have previously tolerated IVIG in the hospital medical day Care Unit.

To study different radiological signs and sequences including apparent diffusion coefficient (ADC) and gradient echo (GRE) to differentiate degenerative parkinsonian syndromes.

Multiple system atrophy (MSA), Parkinson's disease (PD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CbD) differ in the pattern of neurodegeneration and cellular damage. Measuring the ADC, GRE sequences for paramagnetic substances and simple anatomical assessments have been reported individually to assist in separating some of these disorders, but have not been compared.

brain MRIs from May 2002 to February 2008 were retrospectively evaluated by raters blinded to the clinical diagnosis for predefined MRI signs on T1, T2 and GRE sequences. ADC values were quantitatively measured. Medical records were objectively analyzed using standard clinical criteria for different parkinsonian syndromes.

195 cases comprising of 61 PD, 15 MSA-P, 7 MSA-C, 21 PSP, 6 Corticobasal syndrome, 21 not fitting criteria and 64 controls were evaluated. 73% of patients with MSA-P had hypointensity of the putamen (compared to the pallidum) on GRE. The specificity of this sign to diagnose MSA-P was 90% versus PD and 76% versus PSP. When GRE hypointensity was combined with atrophy of the putamen the specificity improved to 98% (versus PD) and 95% (versus PSP) without altering the sensitivity. The ADC values were significantly higher in the middle cerebellar peduncle in cases with MSA-C versus controls, PD and PSP (p<0.001).

The combination of hypointensity and atrophy of the putamen on GRE is useful in differentiating MSA-P from other parkinsonian syndromes.

FOXJ1 is a member of the Forkhead/winged-helix (Fox) family of transcription factors, which is required for the differentiation of the cells acting as adult neural stem cells which participate in neurogenesis and give rise to neurons, astrocytes, oligodendrocytes. The expression pattern of FOXJ1 in the brain after cerebral ischemia has so far not been described. In the current study, we investigated the expression pattern of FOXJ1 in the rat brain after cerebral ischemia by animal model.

We performed a middle cerebral artery occlusion (MCAO) model in adult rats and investigated the expression of FOXJ1 in the brain by Western blotting and immunochemistry; double immunofluorescence staining was used to analyze FOXJ1's co-expression with Ki67.

Western blot analysis showed that the expression of FOXJ1 was lower than normal and sham-operated brain after cerebral ischemia, but the level of FOXJ1 gradually increased from Day 1 to Day 14. Immuohistochemical staining suggested that the immunostaining of FOXJ1 deposited strongly in the ipsilateral and contralateral hemisphere in the cortical penumbra (CP). There was no FOXJ1 expression in the ischemic core (IC). The positive cells in the cortical penumbra might migrat to the ischemic core. In addition, double immunofluorescence staining revealed that FOXJ1 was co-expressed with mAP-2 and gFAP, and Ki67 had the colocalization with NeuN, GFAP, and FOXJ1.

All our findings suggest that FOXJ1 plays an important role on neuronal production and neurogenesis in the adult brain after cerebral ischemia.

Since a large proportion of multiple sclerosis (MS) patients exhibit cognitive deficits, it is important to have reliable and cost-effective screening measures that can be used to follow patients effectively. the objective of this study was to evaluate the clinical value of the Montreal Cognitive Assessment (MoCA) test in detecting cognitive deficits in MS patients.

Forty-one (70.1% women, mean age 44.51 ±7.43) mildly impaired (EDSS: 2.26 ±1.87) MS patients were recruited for this study. In addition to the MoCA, they were administered the MSNQ-P (patient version) and the MSNQ-I (informant version), the bDI-FS and a comprehensive neuropsychological test battery.

there were significant correlations between the MoCA test and the three factors derived from the neuropsychological evaluation (Executive/speed of processing, Learning, Delayed recall). the MoCA test was correlated with the MSNQ-I but only marginally with the MSNQ-P. In addition, there was no significant correlation between the MSNQ-P and the neuropsychological factors, whereas significant correlations were found between two of those factors (Learning and Delayed recall) and the MSNQ-I, suggesting that the informant version is more reliable than the patient version for the presence of cognitive deficits.

the results obtained in the present study support the value of the MoCA test as a screening tool for the presence of cognitive dysfunction in MS patients, even in patients with mild functional disability (EDSS).

Infantile gratification phenomena are self-stimulatory behaviors that are often misdiagnosed as epilepsy. Although the prognosis is thought to be benign, limited long-term follow-up studies exist. This was the objective of our study in addition to exploring the risks of future developmental, behavioral, or neurological abnormalities.

Series of consecutive infants with gratification phenomena were identified both retrospectively and prospectively over an eight year period from a single pediatric neurology service. The diagnosis was based on descriptive history, review of videotaped events, lack of neurological or developmental abnormalities, and normal routine electroencephalogram.

Nineteen infants were followed for 3-11 years (mean 7.1). Their ages ranged between 4-13 months (mean 7) with 79% females. The diagnosis was not reached by the referring physician and 74% were misdiagnosed as epilepsy or movement disorder. The episodes recurred with variable frequency with gradual reduction in number and increase in length of attack-free periods with advancing age. Complete remission was noted in all patients by age 1-3 years (mean 1.9). However, 4 children (21%) developed features of attention deficit hyperactivity disorders (ADHD) on long-term follow up. In this group, the gratification phenomena appeared at a younger age with higher attack frequency.

Gratification phenomena in infants are benign and self-limited, often spontaneously disappearing by two years of age. A correlation with future ADHD was found; however, larger prospective studies are needed to further examine this possible association.