A young man with Guillain-Barré syndrome, with a stroke-like presentation and physical findings suggestive of cervical myelopathy.

We describe the case of a 44-year-old gentleman with hypertension and asthma presenting to the emergency department after noticing right upper-extremity weakness upon awakening. Brain imaging did not reveal a stroke. Initial neurological examination pointed to cervical myelopathy with radiculopathy as well as possible underlying length-dependent peripheral neuropathy as there was right arm strength of 4/5 and there were brisker (3+) reflexes all over except at the right biceps reflex and both ankle reflexes. Cervical spine magnetic resonance imaging (MRI) showed myelomalacia at the C2 level and an os odontoideum (OO). Os odontoideum is a chronic condition that occurs due to the failure of the center of ossification of the dens to fuse with the body of C2. By the next day after a few hours of sustaining a fall, weakness progressed to quadriparesis, without a sensory level on examination, followed by urinary retention. This situation was attributed to a possible cervical cord contusion due to the fall in the presence of OO, with other possibilities being spinal cord hemorrhage, infarct and transverse myelitis. However, repeat scanning of the cervical spine (MRI) did not reveal any acute cord changes. The initial examination for common causes of peripheral neuropathy did not reveal any findings. Finally, the diagnosis of Guillain-Barré syndrome (GBS) was considered, and treatment was initiated with intravenous immunoglobulin. Cerebrospinal fluid analysis was normal. The diagnosis was confirmed using electromyography. Our patient's initial presentation of monoparesis and progression in an asymmetric descending manner was unusual for GBS. His initial presentation mimicked a stroke, and the later progression masqueraded as cervical myelopathy secondary to a chronic cervical cord lesion. The presence of a cervical cord lesion (upper motor neuron) concealed the expected areflexia in GBS. The presence of OO on spine imaging, absence of expected areflexia in GBS, and progression to paraparesis after the fall sidetracked the direction of the initial investigation and led to a relative delay in diagnosis. Nonetheless, appraising the diagnostic data in the clinical context led to an appropriate diagnosis. We emphasize the importance of reconciling the available clinical and diagnostic information to reach the correct diagnosis.

24-Hour video EEG in the evaluation of the first unprovoked seizure.

OBJECTIVE: To assess the gain in detection of epileptiform abnormalities in 24-hour EEG recordings following the first seizure. METHODS: We identified patients who underwent 24-hour video EEG (VEEG) with "first seizure" as an indication. We noted the presence or absence of epileptiform discharges (EDs) in the VEEG study and the latency for the appearance of such discharges. We compared the rate of EDs during the initial 60 min with those occurring only later during the recording. RESULTS: Data from 25 patients, aged 15 to 59, were included. Of the 11 patients with EDs, eight (73%) appeared only after 60 min of recording. This equates to a 32% absolute increase in the detection of EDs across all patients. The latency to first EDs varied from one to 1080 min with a median of 170 min. In four cases, actual subtle seizures were recorded. CONCLUSION: This study suggests an increase in the detection of EDs with the 24-hour studies compared to the traditional shorter recordings, in the context of a first seizure. SIGNIFICANCE: A standard EEG can be performed close to the seizure, followed by a longer up to 24-hour recording if the initial shorter study is unrevealing.

Characteristics of patients with confirmed epilepsy and psychogenic nonepileptic seizures in Qatar.

OBJECTIVE: The Middle Eastern country of Qatar opened its first epilepsy monitoring unit (EMU) in late 2015. This study compared demographic and clinical characteristics of patients with confirmed epilepsy to those of patients with confirmed psychogenic nonepileptic seizures (PNES). METHODS: Data were collected via retrospective chart review on 113 patients admitted for evaluation to the Qatar national health system EMU between November 2015 and May 2017. RESULTS: Seventy-one patients had a confirmed diagnosis (20 had PNES, 46 had epilepsy, 5 had both PNES and epilepsy). Evaluation in 33 patients was inconclusive, and 9 had other medical conditions. Patients with PNES were significantly more likely to be primary Arabic speakers (p = 0.003), and this difference was not explained by education or employment status. The most common referral request in patients with PNES was for recurrent/refractory seizures (p = 0.011), and there was a trend for patients with PNES to have more frequent seizures compared with patients with epilepsy (daily to several per week versus several times a month or less, p = 0.051). Depression was identified in 47% of patients with epilepsy and 65% of patients with PNES, and patients with PNES had higher mean depression scores on the PHQ-9 than patients with epilepsy (p = 0.014). Patients with PNES experienced significantly more fatigue (p = 0.021). Seventy percent of patients with PNES and 50% of patients with epilepsy reported sleep problems. CONCLUSIONS: The characteristics of patients with epilepsy and PNES at the EMU in Qatar were generally similar to those found worldwide. Patients with PNES more often suffered from frequent depression, sleep problems, and fatigue than those with epilepsy, but these were significant concerns for both groups.

No Further Yield of Ambulatory EEG for Epileptiform Discharges Beyond 13 Hours.

INTRODUCTION: This study aimed at evaluating the value added by 24-hour ambulatory EEG (AEEG) by comparing the presence of epileptiform discharges (EDs) between the first 30 minutes of recording versus the following 23.5 hours. MATERIALS AND METHODS: A retrospective review of AEEGs of subjects divided into two groups, epilepsy and undiagnosed episodes of loss of consciousness, was conducted. AEEGs were divided into early EEG (E-EEG) (first 30 minutes) and extended EEG (remaining 23.5 hours). Extended EEGs were further divided into segments (S): 31st minute to 8th hour (SI), 9th to 16th hours (SII) and 17th to 24th hours (SIII). Each consecutive segment was reviewed to identify new EDs not seen previously. RESULTS: Fifty-seven AEEGs were included, the median age being 36.3 years, the range being 18.7 to 78.6 years. There were 38 (66.6%) females. The collective yield of AEEG for detecting EDs was 19/57 (33.4%). The yield of E-EEG of new EDs was 5/57 (9%). During extended EEG, the distribution of EDs was as follows: SI, 12/55 (21.8%); SII, 2/43 (4.6%); and SIII 0/41 (0%). The yield, however, did not increase beyond the 13th hour. In undiagnosed episodes of loss of consciousness group (11), yield was 0/11 in all segments. CONCLUSIONS: (1) There was no value added for yield of EDs by extending the EEG recording beyond 13 hours in epilepsy group. (2) The probability of capturing EDs is negligible if the clinical history does not clearly support the diagnosis of seizure or epilepsy.

Bias in counseling of seizure patients following a transient impairment of consciousness: differential adherence to driver fitness guidelines.

PURPOSE: To determine primary care physicians' counseling as well as patients' driving behaviors following seizure and non-seizure events impairing consciousness in the community. METHODS: Patients attending a rapid-referral first seizure clinic were entered into the study if they were deemed medically-unfit to drive according to national guidelines for driving licensure: had experienced a seizure or an unexplained episode of lost consciousness, and had a valid driver's license at the time of their index event. Risk of physician counseling in the community regarding driving cessation in the interval between initial primary care assessment and neurological consultation was examined as a primary outcome, and patient driving cessation was examined as a secondary outcome. RESULTS: 106 of 192 (55%) patients attending clinic met guideline criteria requiring driver fitness counseling in the primary care community, and 89 patients (46%) were deemed medically-unfit to drive following the initial specialist consultation appointment. Among medically unfit driver cases, 73% were ultimately deemed to have experienced a seizure and 27% had experienced a non-seizure event (e.g. syncope, PNES). Driver fitness counseling was more likely for seizure than non-seizure cases (unadjusted odds ratio: 4.14, p<0.05), as was patient driving cessation (5.10, p<0.05). CONCLUSION: Physician compliance with clinical practice guidelines appears strongly biased when counseling about driving following an episode of transient impairment in consciousness. The failure of the primary care medical community to apply driver fitness counseling equitably to both seizure and non-seizure drivers may have ramifications upon public safety or conversely disease-related quality-of-life.

Counseling at a Seizure Clinic Does Not Ensure Disclosure to the Transportation Registry.

BACKGROUND: The effectiveness of current self-reporting driving laws for medically-unfit potential seizure patients is unknown in Canada. We designed a prospective cohort study of patients' self-reporting practices to the local Transportation Registry (TR) and their driving behaviors following detailed counselling at a seizure clinic in a discretionary physician-reporting jurisdiction. METHODS: Medically unfit drivers, referred to our seizure clinic, who had a valid driver's permit at the time of their episode of impaired consciousness were included. Patients' self-reporting and driving behaviours were assessed using a standardized interview prior to a neurologist's counseling and later at a follow-up visit. RESULTS: Sixty three patients were included; 77% were diagnosed as having had a seizure at the time of their referral. Prior to their seizure clinic visit, 3/63 (5%) had been counseled to self-report to the TR by a non-neurologist physician, and none had done so. Following a neurologist's documented counseling 34/63 (54%) had self-reported themselves at the follow-up seizure clinic visit, and 53/63 (84%) were not driving. CONCLUSION: This prospective study design is the first in North America to examine self-reporting rates for unfit drivers with a seizure disorder. Our findings suggest that self-reporting laws do not ensure high rates of self-reporting behaviors even when patients seen at a seizure clinic are appropriately counseled of their legal obligations. The rate of driving cessation appears greater than the rate of self-reporting to the TR among counseled patients.