Susac syndrome is a rare condition involving the brain, retina, and cochlea. Electroencephalogram (EEG) findings from published case reports show mainly generalized slowing.

A 30-year-old man presented with acute onset of superior vision loss, unsteady gait, and hearing loss. This was accompanied by short-term memory loss and behavioral and mood changes. MRI showed multiple white matter hyperintensities. The EEG showed frontal intermittent rhythmic delta activity. A diagnosis of Susac syndrome was made and treatment with methylprednisolone, ASA, and Nimodipine was instituted. At one-year follow-up, bilateral hearing loss, mild visual impairment, and mood changes were still apparent.

Frontal intermittent rhythmic delta activity can be seen in Susac syndrome.

Accuracy of intracranial magnetic resonance angiography (MRA) and reliability of interpretation are not well established compared to conventional selective catheter angiography. The purpose of this study was to determine the accuracy of MRA in evaluation of intracranial vessels in acute stroke and transient ischemic attack (TIA) patients.

Twenty-nine patients (seven females, 22 males; median age 53) with acute stroke or TIA were enrolled into the study. All patients underwent both MRA using a 3 T clinical magnet and conventional angiography within 48 hours. Median time between MRA and angiography was 263 minutes. Conventional angiography preceded MRA in 15 cases. Fourteen patients received thrombolysis during MRA or angiography. National Institutes of Health Stroke Scale scores were obtained prior to the MR exam. One neuroradiologist rated all conventional angiograms, which were used as gold standard. Five observers, blinded to conventional angiography results and all clinical information except symptom side, rated the MR angiograms. Kappa statistics were used to assess reliability; contingency tables were used to assess accuracy of non-enhanced and enhanced MRA.

Two hundred and fifty two intracranial vessels were assessed. Agreement between raters was good for both non-enhanced (k=0.50) and gadolinium-enhanced (k=0.46) images. There were a total of 26 vessels occluded by DSA. Overall, the non-enhanced MRA showed sensitivity of 84.2% (95% CI 60.4-96.6) and specificity of 84.6% (95% CI 78.6-89.4). The enhanced MRA showed sensitivity of 69.2 (95% CI 38.6-90.9) and specificity of 73.6 (95% CI 65.5-80.7).

Magnetic resonance angiography is a good non-invasive screening tool for assessing intracranial vessel status in acute ischemic stroke. Angiography remains the gold standard for definitive assessment of the intracranial circulation.

We evaluated motor and occipital cortex excitability in migraine patients using transcranial magnetic stimulation.

In this study, we included 15 migraine patients with aura (MwA), 15 patients without aura (MwoA) between attacks, and 31 normal healthy controls. Motor thresholds at rest, amplitudes of motor evoked potentials, central motor conduction time and cortical silent period were measured by stimulation of the motor cortex by using 13.5 cm circular coil and recording from abductor digiti minimi muscle. Additionally, phosphene production and the threshold of phosphene production was determined by stimulation of the visual cortex with the same coil.

No significant differences were observed between the groups with respect to the motor thresholds, Motor evoked potential max/compound muscle action potential max (MEPmax/Mmax) amplitudes, central motor conduction times and duration of cortical silent period. Although not statistically significant, the proportion of the migraineurs with phosphene generation (90%) was found to be higher than that of normal controls (71%). Phosphene threshold levels in migraine patients, however, were significantly lower than those of the controls with MwA patients having the lowest levels.

Our findings indicate that the occipital cortex, but not the motor cortex, is hyperexcitable in migraine patients.

Patients infected with West Nile virus (WNV) may develop acute neurologic disease, which can be severe or even fatal, including WNV meningitis, encephalitis, and an irreversible acute flaccid paralysis or poliomyelitis-like syndrome. Movement disorders have also been described.

We report combined neuronal loss, gliosis, and neurofibrillary tangle formation in the substantia nigra of a 41-year-old man with a history of WNV encephalomyelitis and poliomyelitis-like paralysis.

Clinically our patient did not display parkinsonism, however, it is interesting to speculate whether, in the absence of the residual subacute poliomyelitis-like syndrome, the neuropathologic findings could have eventually evolved clinically into WNV-associated postencephalitic parkinsonism.

SUNCT is a rare primary headache disorder that is associated with activation of the posterior hypothalamus and often poorly responsive to medication. Recently, a relationship between between pituitary microadenoma and SUNCT has been suggested, and reports of both amelioration and exacerbation by dopamine-agonists have been published. These findings suggest a functional role for the hypothalamic-pituitary axis in SUNCT.

We report the long-term 4 year follow-up of a 35 year-old patient with a 14-year history of medically and surgically intractable SUNCT who experienced immediate and complete resolution of symptoms after resection of a 6 mm pituitary microadenoma.

This patient was first seen at the age of 28 years with a 10-year history of attacks of right retro-orbital pain satisfying the IHS criteria for SUNCT. Many medical and surgical treatments were attempted without success. An MRI demonstrated a 6 mm microadenoma without compression of surrounding structures. A trial of bromocriptine caused marked exacerbation of his pain. The patient underwent a trans-sphenoidal resection of the pituitary lesion. SUNCT attacks worsened for the first 24h post-operatively, then disappeared. He has been completely headache-free, without medication, for the past 43 months with the last follow-up being January 2006.

This case emphasizes the relationship between pituitary microadenomas and SUNCT, supports the role of the hypothalamic-pituitary axis in the genesis of SUNCT, and illustrates the importance of careful imaging of the pituitary region in patients with SUNCT.

Episodic ataxia type-2 (EA-2) is an autosomal dominant neurological disorder that has been shown to result from mutations in the CACNA1A gene encoding the P/Q-type calcium channel. Affected individuals experience episodes of cerebellar ataxia usually associated with migraine symptoms, interictal nystagmus, mild residual and in some cases a progressive cerebellar incoordination and respond to acetazolamide treatment. We identified a patient with a positive family history for episodic ataxia, who was originally diagnosed with epilepsy and treated with valproic acid. Subsequent examination revealed that the symptoms were consistent with a diagnosis of EA-2. The patient responded positively to a combination of acetazolamide and valproic acid. Molecular genetic analysis of the CACNA1A gene was performed in order to confirm a diagnosis of EA-2.

The CACNA1A gene was evaluated for mutations using single strand conformational polymorphism analysis and direct DNA sequencing. Allele specific oligo hybridization was used to confirm that the mutation was segregating with only affected family members and was not present in the control group.

In this study we identified a new missense mutation in exon 12 of the CACNA1A gene from a patient with EA-2 whose symptoms could be controlled with a combination of acetazolamide and valproic acid. This G to A transition changes a highly conserved glutamic acid residue to a lysine residue in domain II S2 of the P/Q-type calcium channel α1A subunit.

The use of valproic acid in treating patients with EA-2 is not well documented. Here we describe a patient with a novel mutation in the CACNA1A gene who responded positively to a combination of acetazolamide and valproic acid.

There is evidence that headache response rates may be higher if triptans are used early when a migraine attack is still mild, as compared to when it is treated after pain has reached moderate or severe intensity.

In this randomized, double blind, placebo controlled, parallel group clinical trial, 361 patients took either placebo, sumatriptan 50 mg, or sumatriptan 100 mg in a single attack study. The primary outcome measure was pain-free status at two hours.

In the intention to treat group, two hour pain free rates were 16%, 40%, and 50% in the placebo group, sumatriptan 50 mg group, and the sumatriptan 100 mg group respectively (p<0.001, active treatment groups vs. placebo).

Both sumatriptan 50 mg and 100 mg were significantly superior to placebo for the pain-free end point at two hours. The pain-free response rates in this trial where sumatriptan was taken while the headache was still mild were generally higher than in older clinical trials where headache was treated after reaching a moderate or severe intensity.

A recent report suggested that newly trained Canadian neurosurgeons are experiencing difficulty finding employment in Canada. Such occurrences, in combination with recent certification restrictions imposed in the US, have resulted in increasing concern that we will shortly be seeing a surplus of graduating neurosurgeons in Canada. The purpose of this study was to develop a better understanding of training and employment patterns in the Canadian neurosurgical workforce.

Using a database provided by the Royal College of Physicians and Surgeons of Canada, the current practice location of recent (1990-2002) neurosurgical certificants and a list of all neurosurgeons practicing in Canada were generated. From these data the number of surgeons per 100,000 patient population, and the number of residents required to maintain this workforce were determined.

Practice location could be identified for 183/189 individuals who passed their qualifying examination in neurosurgery during this time. Only 45% of them are currently practicing in Canada. The current service ratio for this specialty is 0.65 per 100,000 population overall. Although 14.6 residents/ year are being trained, only 6.5/ year are required to maintain the existing neurosurgical workforce.

Our data supports the concern about an imminent employment crisis for young neurosurgeons in Canada with more than twice the required number of residents being trained. However, this shortfall of staff positions is at a time when the specialty may be underservicing the country's population. These results highlight the necessity for more cohesive workforce planning in Canada, and in particular, ensuring the appropriate balance between training and need.

Triphasic waves (TWs) and generalized nonconvulsive status epilepticus (GNCSE) share morphological features that may create diagnostic ambiguity.

To describe electroencephalographic differences between TWs and GNCSE.

We retrospectively compared the electroencephalograms (EEGs) of two groups of patients presenting with decreased level of consciousness; those with TWs associated with metabolic encephalopathy and those with GNCSE. We studied the following: demographics, etiology and EEG morphological features. All EEGs were classified blindly (TWs or GNCSE) by two expert EEGers. Agreement between experts and concordance with clinical diagnosis were measured.

We analysed 87 EEGs (71 patients) with TWs and 27 EEGs (13 patients) with GNCSE. Agreement between experts and concordance with clinical diagnosis were excellent. When compared to TWs, epileptiform discharges associated with GNCSE had a higher frequency (mean=2.4Hz vs 1.8Hz) (p<0.001), a shorter duration of phase one (p=0.001), extra-spikes components (69% vs 0%) (p<0.001) and less generalized background slowing (15.1% vs 91.1%) (p<0.001). Amplitude predominance of phase two was common with TWs (40.8% vs 0%) (p=0.01). Lag of phase two was absent in all cases of GNCSE but present in 40.8% of patients with TWs. Noxious or auditory stimulation frequently increased the TWs (51%) while it had no effect on the epileptiform pattern (p=0.008).

Certain EEG morphological criteria and the response to stimulation are very helpful in distinguishing TWs from GNCSE.

The purpose of this study was to develop and validate a self-administered questionnaire to measure the health-related quality of life of patients with pituitary adenoma.

A patient-centred iterative process, involving a literature review and focus group interviews with 84 patients, was used to develop a preliminary 106-item questionnaire and to validate it. The final questionnaire comprised the 30 most important items chosen by 20 patients and 17 items considered important by health care professionals. For assessment of its validity and reliability, 55 patients were asked to complete the final questionnaire, RAND-36, FACT-G/FACT-Br, and Karnofsky Performance Scale (KPS). Concurrent validity of the results of our questionnaire with those of the other instruments was assessed with Pearson correlation coefficients. Known-group validity for the scores of extreme groups was assessed with a Student's t test. Test-retest reliability was determined with Pearson correlation coefficients and a Student's t test for two sets of scores obtained one month apart.

Forty-seven (85.5%) of the 55 questionnaires were completed. Our questionnaire was well correlated with the RAND-36, the FACT-Br, the FACT-G, and the KPS in the general health domain, but not with the KPS overall. Extreme groups (n=20) were significantly different. Test-retest reliability (n=24) was 0.88, and scores one month apart were not significantly different.

Our patient-centred health-related quality of life questionnaire developed for patients with pituitary adenoma had good validity and reliability. This questionnaire could be used as a patient-centred outcome measure in clinical trials and for assessment of disease progression.