Intracranial Hemorrhage in a Patient With Guillain-Barré Syndrome: A Potential Complication, a Coincidental Finding or a Treatment-Related Complication.

This case report demonstrates the difficulty of diagnosing and managing the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS), as well as the rare complication of intracerebral hemorrhage (ICH). A male patient in his mid-60s, presented with bilateral upper limb weakness, bilateral ptosis, and bulbar symptoms. The clinical presentation combined with paraclinical findings supported the diagnosis of PCB. During the course of PCB, the patient required tracheostomy and gastrostomy due to the worsening of his symptoms. Eleven days after hospitalization, and six days after the course of intravenous immunoglobulin (IVIG), the patient developed intracranial bleeding. All clinicians should consider the PCB syndrome in patients with bilateral upper extremity weakness and oropharyngeal involvement, in order to develop a personalized treatment plan and closely monitor potential life-threatening complications such as ICH.

Assessment of the Effect of Erenumab on Efficacy and Quality-of-Life Parameters in a Cohort of Migraine Patients With Treatment Failure in Cyprus.

Erenumab is the first human monoclonal antibody to be approved as a selective therapy for migraine prophylaxis in adults. This study assessed, in a real-world setting, the efficacy of erenumab and its impact on the quality of life (QoL) of Cypriot migraine patients who had failed several treatments in the past. Erenumab was prescribed as a stand-alone or as an add-on therapy to 16 patients with chronic migraine. The first component of the study examined migraine parameters before and after erenumab therapy and included an interim 3-month subjective assessment. In the second component, the patients were asked to complete the validated Migraine-Specific Quality-of-Life Questionnaire-Version 2.1 (MSQ V2.1) during the last month of their individual treatment as a measure of the QoL. The results showed a statistically significant improvement in almost all migraine parameters following erenumab treatment. In the 3-month-interval assessment, 81.3% of the patients reported an improvement in their mental well-being, anxiety, and depression levels, with more than 80% of the patients reporting an improvement in almost all assessed migraine parameters. MSQ V2.1 indicated a good health status in all three domains (mean values > 60 on a scale 0-100), with the "role function preventative" domain having the highest health scores (85). Over a period of 6 months, erenumab was safe, well-tolerated, and effective in preventing migraine symptoms and improving HR-QoL. We conclude that this novel medication, which is not yet part of the national formulary in Cyprus, may be a cost-effective solution in reducing the disease burden of chronic migraine.

Transient, Recurrent Central Nervous System Clinical Manifestations of X-Linked Charcot-Marie-Tooth Disease Presenting with Very Long Latency Periods between Episodes: Is Prolonged Sun Exposure a Provoking Factor?

Charcot-Marie-Tooth disease is one of the most common inherited neurological disorders affecting the peripheral nervous system. The common clinical manifestations of the disease are distal muscle weakness and atrophy, often associated with a characteristic steppage gait and foot deformities. Transient acute and recurrent or chronic central nervous system manifestations, predominantly, dysarthria, dysphagia, motor weakness, and ataxia, have been recognized as a feature of the X-linked type 1 of CMT (CMTX1). The CNS symptoms occur typically in young age and often precede the clinical manifestation of the polyneuropathy. Several predisposing factors such as exercise, fever, and returning from areas of high altitude have been described as triggers of the CNS symptoms; however, in many cases, a substantial cause remains undetermined. In this report, we describe a patient with three attacks of transient CNS deficits at the ages of 11, 21, and 38 years, respectively, which were also accompanied by transient white matter abnormalities on MRI. Two of the attacks occurred after prolonged exposure to sunlight. In our knowledge, this is the first documented case with such long latency periods between CNS attacks as well as the only report describing intense sun exposure as a possible provoking factor.

Spontaneous Intracranial Hypotension Presenting With Frontotemporal Dementia: A Case Report.

Spontaneous intracranial hypotension (SIH) is a rare and often underdiagnosed condition, which commonly results from a cerebrospinal fluid leak. The classic clinical presentation of SIH is a postural headache and dizziness. Less frequent complications include nausea, neck stiffness, and even coma. This case report describes a 70-year-old woman with an initial complaint of postural headaches and sleep attacks, who developed a 22-month progressive history of personality and behavioral changes, cognitive decline, urinary incontinence, chorea, and dysarthria. Although no specific cerebrospinal fluid leak was identified, the patient was suspected of having SIH and her symptoms completely reversed after a 2-month course of steroids. This case highlights that SIH represents a rare and reversible cause of a wide spectrum of neurological symptoms, including dementia. Neurologists should be aware of this diagnosis when evaluating patients with neurological signs and symptoms that cannot otherwise be explained.

A randomized trial for the treatment of refractory status epilepticus.

BACKGROUND: Refractory status epilepticus (RSE) has a mortality of 16-39%; coma induction is advocated for its management, but no comparative study has been performed. We aimed to assess the effectiveness (RSE control, adverse events) of the first course of propofol versus barbiturates in the treatment of RSE. METHODS: In this randomized, single blind, multi-center trial studying adults with RSE not due to cerebral anoxia, medications were titrated toward EEG burst-suppression for 36-48 h and then progressively weaned. The primary endpoint was the proportion of patients with RSE controlled after a first course of study medication; secondary endpoints included tolerability measures. RESULTS: The trial was terminated after 3 years, with only 24 patients recruited of the 150 needed; 14 subjects received propofol, 9 barbiturates. The primary endpoint was reached in 43% in the propofol versus 22% in the barbiturates arm (P = 0.40). Mortality (43 vs. 34%; P = 1.00) and return to baseline clinical conditions at 3 months (36 vs. 44%; P = 1.00) were similar. While infections and arterial hypotension did not differ between groups, barbiturate use was associated with a significantly longer mechanical ventilation (P = 0.03). A non-fatal propofol infusion syndrome was detected in one patient, while one subject died of bowel ischemia after barbiturates. DISCUSSION: Although undersampled, this trial shows significantly longer mechanical ventilation with barbiturates and the occurrence of severe treatment-related complications in both arms. We describe practical issues necessary for the success of future studies needed to improve the current unsatisfactory state of evidence.

Cerebral embolism during elective carotid endarterectomy treated with tissue plasminogen activator: utility of intraoperative EEG monitoring.

Electroencephalography (EEG) is routinely used during elective carotid endarterectomy (CEA) for monitoring cerebral perfusion. The period most frequently associated with cerebral hypoperfusion is the one during the clamping of the carotid artery. We present a case whereby acute hypoperfusion, as detected by ipsilateral hemispheric slowing and attenuation of the fast frequencies on EEG, was detected in the period prior to clamping of the carotid artery. The acute changes were caused by a cerebral embolism. Following emergent treatment with intraoperative thrombolytic therapy with intra-arterial tissue plasminogen activator (t-PA) the EEG changes reversed fully. We discuss the utility of intraoperative EEG monitoring in the detection and treatment of cerebral embolism. The ability of EEG to intraoperatively measure the function of the at-risk cerebral cortex makes it not only a useful tool in detecting acute changes such as from a large embolism, but also in guiding necessary treatment by offering direct feedback in the absence of reliable imaging and clinical examination.

The effects of EEG suppression and anesthetics on stimulus thresholds in functional cortical motor mapping.

OBJECTIVE: To investigate the effects of EEG suppression and anesthetics on variability of electrical stimulus thresholds during functional cortical motor mapping, and the possible influences of age, lesion location and pathology. METHODS: Multivariate regression analysis was performed to study these relationships in 60 cases of successful mapping using the monopolar multipulse train technique. RESULTS: An increase in the length of EEG "flats" by 1s produced an increase in stimulus threshold by 1.08 mA (p=0.0004). Administration of TIVA (total intravenous anesthesia) or inhalational agents produced an additional increase in threshold by 1.27 mA (p=0.38) or 4.84 mA (p=0.04) respectively, when compared to awake patients. CONCLUSIONS: Depth of cortical suppression impacts the stimulus thresholds. The effect of TIVA on thresholds is mediated by its effect on cortical excitability. The effects of inhalational agents on thresholds involve their influence on excitability at other levels of the neuraxis. SIGNIFICANCE: The study represents an important step towards building a predictive model for stimulus thresholds. It also improves our understanding of the relationships of anesthetics, EEG burst suppression pattern and age with cortical excitability.

Status Epilepticus Severity Score (STESS): a tool to orient early treatment strategy.

BACKGROUND: Status epilepticus (SE) treatment ranges from small benzodiazepine doses to coma induction. For some SE subgroups, it is unclear how the risk of an aggressive therapeutic approach balances with outcome improvement. We recently developed a prognostic score (Status Epilepticus Severity Score, STESS), relying on four outcome predictors (age, history of seizures, seizure type and extent of consciousness impairment), determined before treatment institution. Our aim was to assess whether the score might have a role in the treatment strategy choice. METHODS: This cohort study involved adult patients in three centers. For each patient, the STESS was calculated before primary outcome assessment: survival vs. death at discharge. Its ability to predict survival was estimated through the negative predictive value for mortality (NPV). Stratified odds ratios (OR) for mortality were calculated considering coma induction as exposure; strata were defined by the STESS level. RESULTS: In the observed 154 patients, the STESS had an excellent negative predictive value (0.97). A favorable STESS was highly related to survival (P < 0.001), and to return to baseline clinical condition in survivors (P < 0.001). The combined Mantel-Haenszel OR for mortality in patients stratified after coma induction and their STESS was 1.5 (95 % CI: 0.59-3.83). CONCLUSION: The STESS reliably identifies SE patients who will survive. Early aggressive treatment could not be routinely warranted in patients with a favorable STESS, who will almost certainly survive their SE episode. A randomized trial using this score would be needed to confirm this hypothesis.

Tolerability and dosing experience of intravenous levetiracetam in children and infants.

OBJECTIVE: To evaluate the use and tolerability of intravenous (IV) levetiracetam (LEV) in a pediatric cohort under 14 years of age. METHODS: A retrospective analysis of the use of the IV formulation of LEV was performed for the first 9 months that it was available in our institution. RESULTS: Overall, 118 infusions in 15 patients were performed during the period assessed. No adverse reactions were observed during the infusion phase of the IV formulation. Nine minor adverse reactions were observed during the rest of the hospitalization that were potentially related to the IV formulation. Three of these were decreases in the white cell counts, while the majority of the rest were behavioral adverse effects. The median starting dose in LEV naïve patients was 8.8 mg/kg (range=5.0-50), with a median maintenance dose of 30.4 mg/kg/day for all patients (range=5.0-92). The majority of these infusions (82/118) were in a subgroup of children under 4 years of age where the median maintenance dose was 28 mg/kg/day. CONCLUSION: IV LEV was very well tolerated in this cohort of pediatric patients including a subgroup of children under the age of 4 years.