Osteomalacia is an uncommon cause of muscle weakness. Our objectives were to describe features of myopathy associated with Vitamin D deficiency and examine the contributing factors leading to osteomalacic myopathy in our region.

Patients identified retrospectively for the six year period ending in December 2006 with the diagnosis of osteomalacia and/or Vitamin D deficiency associated proximal muscle weakness were included. They were followed in three major centers in western Saudi Arabia. Clinical, biochemical, radiological, and electrophysiological findings were collected before and after Vitamin D treatment by chart review.

Forty seven female patients aged 13-46 years (mean 23.5, SD 4.5) were included. All were veiled and covered heavily when outside the house for social and cultural reasons. Only eight (17%) had adequate varied diet with daily milk ingestion. All patients presented with progressive proximal muscle weakness lasting 6-24 months (mean 14) prior to our evaluation. The weakness was severe in six (13%) patients leading to wheel chair bound states. Associated musculoskeletal pain involving the back, hips, or lower limbs was common (66%). Osteomalcia was the referral diagnosis in only 11 patients and the remaining 36 (77%) patients were misdiagnosed. All patients had metabolic and radiological profiles suggestive of osteomalacia. Remarkable recovery was documented in all patients following oral cholecalciferol and calcium supplementation.

Vitamin D deficiency is an important treatable cause of osteomalacic myopathy in Saudi Arabia. The diagnosis is frequently delayed or missed. Screening for Vitamin D deficiency in patients with acquired myopathy is needed to identify this treatable disorder.

Neurovascular compression (NVC) may cause cranial mononeuropathy but lacks a definitive diagnostic investigation. We hypothesized that the arterial pressure wave (APW) would interact at the neurovascular interface in NVC to inhibit transmission of transcranial magnetic stimulation (TMS) stimuli to affected muscles.

We report a novel neurophysiological method coupling cardiovascular physiology with TMS. The electrocardiogram (ECG) and arterial pressure wave (APW) were coupled to triggering of cortical TMS in a patient with NVC-induced spinal accessory (CNXI) mononeuropathy. Outcome measures included motor evoked potential (MEP) amplitudes and firing probabilities of normal and affected trapezieus (TPZ). Values at intervals in proximity to the APW (40/80/120/160ms) were compared to baseline (800ms) using ANOVA and student t-test.

Electrocardiogram triggered TMS of CNXI pathways with 100% reliability. MEP amplitudes were decreased in proximity to the APW, particularly at 120ms (0.21±0.04 mV versus 0.39±0.10mV, p=0.003). TPZ firing probabilities were similarly inhibited (43.8% versus 88.2%, p=0.009). No effect of APW proximity was observed on the unaffected side (p=0.868). Procedures were well tolerated.

Vascular compression causes CNXI mononeuropathy. Transcranial magnetic stimulation-cardiovascular coupling may evaluate neurovascular junction interactions and non-invasively diagnose NVC.

Head injury causes substantial morbidity and mortality in children. The ThinkFirst For Kids (TFFK) program improves knowledge of safe behaviours in kindergarten to Grade 6 students.

This study evaluated the TFFK curriculum for grade 7/8 students. Knowledge acquisition was assessed quantitatively by an injury prevention test at baseline, at curriculum completion, and six weeks later. Participant experiences and behaviours were explored qualitatively by interviews and focus groups.

Students (n=204) and teachers (n=6) from four schools participated in this study. Test scores improved from baseline (26.48±0.17, n=204), to completion (27.75±0.16, n=176), to six weeks post-completion (28.65±0.13, n=111) (p<0.05). Most students reported their decision-making and participation in risky behaviors was altered by the curriculum.

The TFFK curriculum may promote education about head injury prevention among Grade 7/8 students, with a suggestion of long-term knowledge retention. The curriculum was wellreceived and may be suitable to reduce risky behavior and injuries in children.

Prior studies have shown that the electroencephalogram (EEG) is of low diagnostic yield in the evaluation of syncope but have not looked at the yield according to referring physician specialty. The goals of this study were to determine if the yield of the EEG is higher when ordered by neurologists and whether EEGs with abnormal findings resulted in any significant change in patient management.

We retrospectively reviewed the records of the EEGs requested for a clinical diagnosis of syncope, convulsive syncope, loss of consciousness, or falls from 2003 to 2007 at our institution. We obtained further information from the medical record of patients with an abnormal EEG.

Of 517 EEGs meeting our inclusion criteria, only 57 (11.0%) were read as abnormal. No EEG was positive for epileptiform activity and only 9 (1.6%) showed potentially epileptic activity. EEGs ordered by neurologists did not have a higher yield compared to non-neurologists. Five abnormal EEGs resulted in further investigations being ordered. One patient was ultimately started on phenytoin.

EEGs requested for the evaluation of patients with suspected syncope have an extremely low diagnostic yield and do not significantly alter the management of the patients, regardless of the specialty of the referring physician.

To explore the influence of environmental conditions on pattern visual evoked potential (VEP) recordings.

Fourteen subjects with no known ocular pathology were recruited for the study. In an attempt to optimize the recording conditions, VEP recordings were performed in both the seated and recumbent positions. Comparisons were made between recordings using either LCD or CRT displays and recordings obtained in silence or with quiet background music. Paired recordings (in which only one variable was changed) were analyzed for changes in P100 latency, RMS noise, and variability.

Baseline RMS noise demonstrated a significant decrease in the variability during the first 50msec accompanied by a 73% decrease in recording time for recumbent position when compared to the seated position (p<0.05). Visual evoked potentials recorded using LCD monitors demonstrated a significant increase in the P100 latency when compared to CRT recordings in the same subjects. The addition of background music did not affect the amount of RMS noise during the first 50msec of the recordings.

This study demonstrates that the use of the recumbent position increases patient comfort and improves the signal to noise ratio. In contrast, the addition of background music to relax the patient did not improve the recording signal. Furthermore, the study illustrates the importance of avoiding low-contrast visual stimulation patterns obtained with LCD as they lead to higher latencies resulting in false positive recordings. These findings are important when establishing or modifying a pattern VEP recording protocol.

The purpose of this study was to develop and validate a self-administered questionnaire to measure the health-related quality of life (QOL) of patients with brain cancer. We wanted to assess both core and disease-specific concerns in a single, easy-to-use instrument, thus promoting concision and clinical utility. The questionnaire departs from its predecessors in that it was designed for- and validated among French speaking Canadians.

A focus group of health professionals was used to develop items for the questionnaire, which was later validated with 105 patients suffering from brain cancer. The underlying structure of the questionnaire was investigated using principal component analysis and confirmed using a principal factor analysis.

The final version of the questionnaire contains 30 items. Seven multi-item scales, tapping into distinct dimensions of QOL, were uncovered (i.e., functional well-being, symptom severity/fear of death, social support/acceptance of disease, autonomy in personal care, digestive symptomatology, neurocognitive function, and pain). Assessment of reliability revealed elevated internal consistency for each of the seven scales (Cronbach coefficient alpha ≥≥.65), whereas known-groups validity (anchor-based approach) revealed that the different dimensions uniquely discriminated between patients with different functional levels (Karnofsky Performance Scores) and clinical status (exposure to neurosurgery, radiotherapy, and use of chemotherapy and anticonvulsants).

Our QOL questionnaire, the Sherbrooke Neuro-Oncology Assessment Scale, or SNAS, taps into both core and disease-specific issues relevant to neuro-oncology patients. It has good validity and reliability, and clearly reflects the multidimensional nature of QOL. Depending on the research focus, it may be used in clinical trials to track the impact of disease and/or treatment on satisfaction, functional status, and general well-being.

Perimesencephalic subarachnoid hemorrhage (PSH) is a relatively benign clinical entity with a low risk of recurrent bleeding. The precise etiology of PSH has not yet been determined. We report here three cases of PSH with clinical and radiological features that support a venous system as a cause.

The first patient, a 72-year-old woman, had PSH and venous hemorrhagic infarct in the left thalamus on non-contrast CT. Subsequent cerebral angiography revealed widespread thrombosis in the cerebral venous system, a potential cause for reflux overflow hemorrhage. The second patient, a 55-year-old man with an established diagnosis of neuro-Behçet's disease, a well-known cause for cerebral venulitis, presented with PSH one year later. The third patient, a 39-year-old female, with incomplete Behçet's disease was admitted with PSH.

Current concepts on the anatomic origin and the possible pathophysiologic mechanism leading to PSH are discussed. The underlying pathological conditions in the venous system in our cases provide theoretical clues to the anatomic origin of PSH in general.

Resiniferatoxin, the most potent agonist of inflammatory pain/vanilloid receptor/cation channel (TRPV1) can be used for neuron subtype specific ablation of pain generating cells at the level of the peripheral nervous system by Ca2+-excytotoxicity. Molecular neurosurgery is an emerging technology either to alleviate severe pain in cancer or treat/prevent different local neuropathies. Our aim was determining sensory modalities that may be lost after resiniferatoxin treatment.

Newborn or adult mice were treated with resiniferatoxin, then changes in chemical and heat sensitivity were correlated with alterations of the cell composition of sensory ganglions.

Only mice treated at adult age became less sensitive to heat stimuli, while both treatment groups lost sensitivity to specific vanilloid agonists of TRPV1 and, interestingly, to allyl-isothiocyanate, a selective agonist of TRPA1. Our in vivo and post mortem analytical results confirmed that TRPV1 and TRPA1 function together and resiniferatoxin-mediated neurosurgery removes both sensor molecules

In adult mice resiniferatoxin causes: i) desensitization to heat and ii) sensitization to cold. Cold hyperalgesia, an imbalance in thermosensation, might be conferred by a prominent cold receptor that is expressed in surviving resiniferatoxin-resistant sensory neurons and compensates for pain signals lost with TRPA1 and TRPV1 double positive cells in the peripheral nervous system.

Sema4D/CD100 is a type of class 4 semaphorin, exhibiting crucial rôles in growth cone guidance in developing neurons. Sema4D is widely expressed throughout the central nervous system in embryonic mouse brain, and is selectively localized to oligodendrocytes and myelin in the postnatal brain. However, direct evidence of the actual involvement of Sema4D in the neuronal network development crucial for neurobehavioral performance is still lacking. The present study therefore examined whether Sema4D deficiency leads to abnormal behavioral development.

Both wild-type and Sema4D-deficient mice were subjected to behavioral analyses including open-field, adhesive tape removal, rotarod tests and a water maze task.

Open-field tests revealed increased locomotor activity in Sema4D-deficient mice with less percentage of time spent in the center of the field. In both the adhesive tape removal and rotarod tests, which examine motor coordination and balance, Sema4D-deficient mice showed significantly superior performance, suggesting facilitated motor behavior. Both Sema4D-deficient and wild-type mice successfully learnt the water maze task, locating a hidden escape platform, and also showed precise memory for the platform position in probe tests. However, the swimming speed of Sema4D-deficient mice was significantly faster than that of wild-type mice, providing further evidence of their accelerated motor behavior.

Our mouse behavioral analyses revealed enhanced motor activity in Sema4D-deficient mice, suggesting the crucial involvement of Sema4D in the neurodevelopmental processes of the central structures mediating motor behavior in mice.

To predict success of endoscopic third ventriculostomy (ETV) in patients with obstructive hydrocephalus, we evaluated pre- and post- operative phase-contrast cine magnetic resonance images (PC MRI) on cerebrospinal fluid (CSF), cine flow in basal cisterns around the brain stem, and cervical medullar junction (CMJ) retrospectively.

The study involved 102 patients with mean age of 36.74±23.25, and F/M = 1.55. All patients had PC MRI taken both pre- and post-operatively. A dynamic MRI video of PC MRI was reviewed at sagittal, axial, and coronal sections to determine cistern flows around the brain stem and CMJ. For quantitative analysis, quadrants were divided around midbrain axially to evaluate interpeduncular, quadrageminal, and ambient cisterns of both sides using scores of 0 to 60. Pre- and post- pontine and CMJ flows were shown in sagittal view and scored 0 to 20, and lateral cerebellopontine cisterns of both sides were measured on coronal image and scored 0 to 20.

No significant difference in CSF flow was seen from three individual views and total cine score, or between ETV success and failure groups by multivariate analysis of variance. Kaplan-Meier Analysis and Spearman's Correlation Test produced no relationship between MRI cine flow scores and interval period after surgery to ETV failure.

PC MRI cine flow failed to demonstrate significant differences between successful and failed ETV groups. This indicates in addition to achieving an adequate fenestration, CSF pathways beyond the basal cisterns around the brain stem and CMJ may play an essential role in achieving ETV success.