Pedro Viana

Familial SUNCT in mother and son

Background Trigeminal autonomic cephalalgias comprise a heterogeneous group of lateralized headaches associated with ipsilateral autonomic symptoms. They are usually localized within the territory of one or more rami of the trigeminal nerve, but may be localized outside its cutaneous territory. Although these headaches are considered primary disorders, the evidence supporting their genetic nature is lacking, particularly concerning their neuralgic forms, with the exception of a familial case described partly based on a historical account. Case reports We report on a mother and son with episodic, short-lasting, intense, paroxysmal headaches, with the same localization in the left retroauricular region, associated with prominent conjunctival injection and tearing, which are consistent with the diagnosis of SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing). Discussion These cases corroborate the existence of hereditary forms of this disorder, thus supporting its primary nature.

InMS: Chronic insomnia disorder in multiple sclerosis – a Portuguese multicentre study on prevalence, subtypes, associated factors and impact on quality of life

BACKGROUND Sleep may be disrupted in Multiple Sclerosis (MS), but the prevalence of chronic insomnia disorder (CID) using standard diagnostic criteria is unknown. OBJECTIVES To determine the prevalence of CID in an MS population, the frequency of CID subtypes, associated factors and impact on quality of life (QoL). METHODS Multicentre, hospital-based cross-sectional study. An adapted version of the Brief Insomnia Questionnaire was applied to a consecutively recruited MS population. The influence of demographic, MS-related features, fatigue, medical and psychiatric comorbidities, nocturnal symptoms, other sleep disorders, dysfunctional beliefs about sleep in CID was evaluated. The relation between CID and QoL was analysed. RESULTS Of 206 MS patients, 22.3% fulfilled criteria for CID, with initial insomnia in 11.7%, maintenance insomnia in 11.2% and terminal insomnia in 10.2% of patients. CID was more frequent in female patients, those with nocturnal symptoms, medical comorbidities, higher levels of anxiety, depression and fatigue. Multivariable analysis identified female sex, medical comorbidities, anxiety and fatigue as independent factors for CID. CID patients had a significantly lower self-reported QoL. CONCLUSIONS CID is prevalent in MS patients and associated with psychiatric and medical comorbidities, as well as fatigue. It has a negative impact on QoL.

Epilepsia partialis continua after an anterior circulation ischaemic stroke

Although cerebrovascular disorders are the main cause of epilepsia partialis continua (EPC) in adulthood, the frequency of EPC after stroke is unknown. The aim was to prospectively ascertain its frequency 1 year after an ischaemic stroke.

Seizures, electroencephalographic abnormalities, and outcome of ischemic stroke patients

Seizures and electroencephalographic (EEG) abnormalities have been associated with unfavorable stroke functional outcome. However, this association may depend on clinical and imaging stroke severity. We set out to analyze whether epileptic seizures and early EEG abnormalities are predictors of stroke outcome after adjustment for age and clinical/imaging infarct severity.

Management of Neurologic Manifestations in Patients with Liver Disease

Opinion statementLiver disease, both in its acute and chronic forms, can be associated with a wide spectrum of neurologic manifestations, both central and peripheral, ranging in severity from subclinical changes to neurocritical conditions. Neurologists are frequently consulted to participate in their management. In this review, we present an overview of management strategies for patients with hepatic disease whose clinical course is complicated by neurologic manifestations. Type A hepatic encephalopathy (HE), which occurs in acute liver failure, is a neurologic emergency, and multiple measures should be taken to prevent and treat cerebral edema. In Type C HE, which occurs in chronic liver disease, management should be aimed at correcting precipitant factors and hyperammonemia. There is an increasing spectrum of drug treatments available to minimize ammonia toxicity. Acquired hepatocerebral degeneration is a rare complication of the chronic form of HE, with typical clinical and brain MRI findings, whose most effective treatment is liver transplantation. Epilepsy is frequent and of multifactorial cause in patients with hepatic disease, and careful considerations should be made regarding choice of the appropriate anti-epileptic drugs. Several mechanisms increase the risk of stroke in hepatic disease, but many of the drugs used to treat and prevent stroke are contraindicated in severe hepatic failure. Hepatitis C infection increases the risk of ischemic stroke. Hemorrhagic stroke is more frequent in patients with liver disease of alcoholic etiology. Viral hepatitis is associated with a wide range of immune-mediated complications, mostly in the peripheral nervous system, which respond to different types of immunomodulatory treatment. Several drugs used to treat hepatic disease, such as the classical and the new direct-acting antivirals, may have neurologic complications which in some cases preclude its continued use.

A peripheral pathway to restless legs syndrome? Clues from familial amyloid polyneuropathy

BACKGROUND The relationship between restless legs syndrome (RLS) and peripheral neuropathy remains unclear. In order to clarify this relationship, we investigated if RLS is increased in familial amyloid polyneuropathy related to transthyretin (TTR-FAP) and investigated factors associated with RLS in this population. METHODS RLS frequency was compared between TTR-FAP patients and controls. Secondly, TTR-FAP patients with and without RLS were compared regarding demographic and clinical characteristics. RESULTS RLS frequency was significantly increased in TTR-FAP, with 18/98 (18.4%) cases contrasting with 5/104 (4.8%) controls (p-value 0.002). This difference remained significant after adjusting for confounders. In TTR-FAP patients, female sex (p-value 0.037), obesity (p-value 0.036) and weight excess (p-value 0.048) were associated with RLS, contrary to other classical RLS risk factors. CONCLUSIONS RLS frequency is increased in TTR-FAP, thus supporting an association between RLS and neuropathy. This may represent a peripheral pathway in RLS pathogenesis. Furthermore, our results suggest that female sex and obesity/weight excess may be risk factors for RLS development among TTR-FAP patients.

Usefulness of EEG for the differential diagnosis of possible transient ischemic attack

Highlights • FSWA was the commonest EEG abnormality in patients with possible TIA.• FSWA in the early EEG did not distinguish TIA from epileptic seizure patients.• The majority of epileptic seizure patients did not have EA in the early or late EEG.• In patients with seizures, FSWA in the early EEG was more common than EA.• FSWA in late EEG was more likely in patients with an epileptic seizure than with a TIA.

Cortical myoclonus during IV thrombolysis for ischemic stroke.

We describe a patient with an acute middle cerebral artery ischemic stroke developing subtle involuntary movements of the paretic upper limb with cortical origin during rt-PA perfusion. Despite the multiple potential pathophysiological mechanisms for the relationship between thrombolysis and epileptic activity, seizures during this procedure are scarcely reported. Our hypothesis is that subtle and transient clinical seizures, like those described in our patient, may not be detected or are misdiagnosed as nonepileptic involuntary movements. We aimed to draw attention to the recognition challenge of this paroxysmal motor behavior, highlighting this clinical and neurophysiological identification using video recording and back-average analysis of the EEG.

“Cough Hemicrania” – An Overlapping Form of Headache: Case Reports

To describe two patients with recurring unilateral brief headaches that fulfilled criteria for both primary cough headache (CH) and chronic paroxysmal hemicrania (CPH).

Quantitative EEG and functional outcome following acute ischemic stroke

OBJECTIVE To identify the most accurate quantitative electroencephalographic (qEEG) predictor(s) of unfavorable post-ischemic stroke outcome, and its discriminative capacity compared to already known demographic, clinical and imaging prognostic markers. METHODS Prospective cohort of 151 consecutive anterior circulation ischemic stroke patients followed for 12 months. EEG was recorded within 72 h and at discharge or 7 days post-stroke. QEEG (global band power, symmetry, affected/unaffected hemisphere and time changes) indices were calculated from mean Fast Fourier Transform and analyzed as predictors of unfavorable outcome (mRS ≥ 3), at discharge and 12 months poststroke, before and after adjustment for age, admission NIHSS and ASPECTS. RESULTS Higher delta, lower alpha and beta relative powers (RP) predicted outcome. Indices with higher discriminative capacity were delta-theta to alpha-beta ratio (DTABR) and alpha RP. Outcome models including either of these and other clinical/imaging stroke outcome predictors were superior to models without qEEG data. In models with qEEG indices, infarct size was not a significant outcome predictor. CONCLUSIONS DTAABR and alpha RP are the best qEEG indices and superior to ASPECTS in post-stroke outcome prediction. They improve the discriminative capacity of already known clinical and imaging stroke outcome predictors, both at discharge and 12 months after stroke. SIGNIFICANCE qEEG indices are independent predictors of stroke outcome.