Zagorka Jovanovic

Medication overuse headache: clinical features predicting treatment outcome at 1-year follow-up.

We present a prospective study of 240 patients with medication overuse headache (MOH) treated with drug withdrawal and prophylactic medications. At 1-year follow-up, 137 (57.1%) patients were without chronic headache and without medication overuse, eight (3.3%) patients did not improve after withdrawal and 95 (39.6%) relapsed developing recurrent overuse. Age at time of MOH diagnosis, regular use of benzodiazepines, frequency and Migraine Disability Assessment (MIDAS) score of chronic headache, age at onset of primary headache, frequency and MIDAS score of primary headache, ergotamine compound overuse and daily drug intake were significantly different between successfully and unsuccessfully treated patients. Multivariate analysis determined the frequency of primary headache disorder, ergotamine overuse and disability of chronic headache estimated by MIDAS as independent predictors of treatment efficacy at 1-year follow-up.

Cerebral Small Vessel Disease in Pseudoxanthoma Elasticum: Three Cases

BACKGROUND Cerebral small vessel disease is rarely described in association with pseudoxanthoma elasticum (PXE), a hereditary connective tissue disorder with skin, eye and vascular manifestations. This autosomally inherited elastic tissue disease has been attributed to mutations in the ABCC6 gene located on chromosome 16p13.1. Different stroke mechanisms are suggested in PXE patients, arterial hypertension and accelerated atherosclerosis being the leading ones. CASE DESCRIPTIONS Case 1: A 49-year-old man with history of mild hypertension presented with recurrent transient ischemic attacks. At the age of 42, evaluation for progressive visual loss and skin changes led to diagnosis of PXE. Brain magnetic resonance imaging (MRI) disclosed multiple lacunar infarctions and confluent periventricular white matter lesions (WML). Case 2: A 71-year-old woman with history of mild hypertension suffered right-sided stroke. Diagnosis of PXE was made at the age of 48 due to severe visual loss and skin changes. Brain MRI revealed multiple lacunar infarctions and subcortical ischemic leukoencephalopathy. Case 3: A 47-year-old woman with prominent skin changes and bilateral amblyopia developed right-sided weakness. Skin biopsy confirmed PXE. Several lacunar infarcts in deep white matter and pons were revealed on MRI. DISCUSSION We present three patients with clinical and histopathological features of PXE who presented with multiple lacunar strokes, two with extensive confluent WML. These cases illustrate that PXE is a rare but significant risk factor for small vessel disease and stroke in patients of all age groups. Occlusive small vessel disease and subsequent lacunar infarcts and WML represent important PXE manifestations.

Cluster headache and paroxysmal hemicrania: differential diagnosis

The utility of the differences between cluster headache (CH) and paroxysmal hemicrania (PH) is limited by the considerable overlap of their clinical characteristics. We compared 54 patients with CH and eight patients with PH in terms of demographic features, characteristics of headache attacks, associated autonomic features, temporal forms of disorders, and response to verapamil. According to our results, clinical features that distinguished CH and PH patients were: maximal pain localization, ocular in CH patients and extra-ocular in PH group; mean attack duration was longer and mean attack frequency was lower in CH patients in comparison with PH patients. Conjuctival injection was the only autonomic sign seen more frequently in CH patients. There were more CH patients with episodic and more PH patients with unremitting form of the disorder in examined groups. Although statistical analysis pointed out a significant difference between these clinical features, there was no clinical characteristic that exclusively belonged to one of these headache entities. Demographic characteristics (age, gender, social background), the other headache attack features (nocturnal attacks, interattack tenderness), the other autonomic signs, as well as the response to verapamil did not differ significantly between two groups.

Vertebral Artery Vascular Loop in SUNCT and Concomitant Trigeminal Neuralgia. Case report

Trigeminal neuralgia (TN) is the most important syndrome among the pain disorders involving the fifth cranial nerve. Although the underlying mechanism of TN remains partially unknown, the effects of direct stimulation by closely located blood vessels are the most common related abnormalities in TN (1). Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) represents a brief headache syndrome first described in 1989 (2). It has been grouped with other shortduration headache syndromes with prominent autonomic features under the general term of trigeminal autonomic cephalalgias (TAC) (3). Coexistence of TAC and TN was described for cluster headache and paroxysmal hemicrania and they were accepted as separate entities by the new International Headache Society (IHS) classification (4). However, patients with overlapping SUNCT and TN have been described and, according to the IHS classification, these patients should receive separate diagnoses. We report a male patient who had 6-month history of simultaneous occurrence of two types of headaches: TN of the first branch and SUNCT. Neuroimaging studies revealed a ‘vascular loop’ of the right vertebral artery compressing the left trigeminal root zone entry. Both types of headaches were successfully treated with gabapentin, leaving the patient attack free during a follow-up of 8 months.

Baseline characteristic of patients presenting with lacunar stroke and cerebral small vessel disease may predict future development of depression

Cerebral small vessel disease (SVD) is associated with late‐onset depression and increases the risk for depression after stroke. We aimed to investigate baseline predictors of depression after long‐term follow‐up in patients with SVD, initially presenting with first‐ever lacunar stroke, free of depression and cognitive impairment.

Brain sonography insight into the midbrain in myotonic dystrophy type 2

Introduction: The aim of this study was to analyze transcranial sonography (TCS) findings in genetically confirmed myotonic dystrophy type 2 (DM2) patients. Methods: Forty DM2 patients and 38 gender‐ and age‐matched healthy controls (HCs) underwent TCS through the pre‐auricular acoustic bone window. Results: Substantia nigra hyperechogenicity was found in 20% of DM2 patients compared with 3% of HCs. Brainstem raphe (BR) hypoechogenicity was more common in DM2 patients compared with HCs (56% vs. 10%, P < 0.01), and it was more common in patients with fatigue and excessive daytime sleepiness (P < 0.05). Diameter of the third ventricle was increased in DM2 patients compared with HCs (5.8 ± 1.7 vs. 5.1 ± 1.0 mm, P < 0.05). Conclusions: Finding BR hypoechogenicity might have clinical implication because of the potential response to serotonin‐reuptake inhibitors. TCS revealed alterations in brain structures previously not seen in MRI studies. Muscle Nerve 53: 700–704, 2016

Do interictal microembolic signals play a role in higher cortical dysfunction during migraine aura

Introduction The aim of this study was to evaluate the prevalence and clinical impact of interictal microembolic signals (MES) in patients suffering from migraine with higher cortical dysfunction (HCD), such as language and memory impairment, during an aura. Patients and methods This study was carried out on 34 migraineurs with language and memory impairment during aura (HCD group), 31 migraineurs with only visual or visual and somatosensory symptoms during aura (Control group I), and 34 healthy controls (Control group II). We used a Doppler instrument to detect microemboli. Demographic data, disease features and the detection of MES between these groups, as well as the predictors of HCD during the aura, were analyzed. Results The duration of aura was longer and the frequency of aura was higher among patients with language and memory impairment during aura compared to Control group I. MES was detected in 29.4% patients from the HCD group, which was significantly higher compared to 3.2% in Control group I and 5.9% in Control group II. Regarding the absence or presence of MES, demographic and aura features were not different in the HCD subgroups. A longer duration of aura, the presence of somatosensory symptoms during the aura and the presence of interictal MES were independent predictors of HCD during the aura. Conclusion The present findings indicate that HCD and MES are related in patients with migraine with aura. Further research is needed to better understand the exact pathophysiological mechanism.

What are the differences between younger and older patients with symptomatic small vessel disease

OBJECTIVE Although typically linked to aging, small vessel disease (SVD) is also observed in younger adult patients, with common vascular risk factors (RF). We aimed to investigate features of SVD occurrence at an early adult age. PATIENTS AND METHODS Vascular RF, functional and cognitive status and severity of lesions on MRI expressed as total score on Age-Related White Matter Changes (ARWMC) scale were analyzed in 200 consecutive patients with cerebral SVD admitted to a tertiary neurological hospital. Variables were compared between younger (35-55 years) and older (>56 years) patients. RESULTS In this study, 63 (31.5%) of patients were 55 years or younger. Both age groups had comparable RF profiles, but smoking emerged as an independent predictor for SVD at a younger age (OR 2.9; 95% CI 1.5-5.5; p=0.002). Younger patients had better functional (OR 1.8; 95% CI 1.3-2.5; p=0.0001) and cognitive (χ(2) 13.94; p=0.0009) status compared to older patients. However, two thirds of younger patients had some degree of cognitive deficit. Total score on ARWMC scale was lower in younger patients (mean 12.3 in younger versus 15.2 in older, OR 1.11; 95% CI 1.0-1.18; p=0.001). There was a strong correlation in both groups between functional score, cognitive status and ARWMC score (p<0.0001). CONCLUSION In our dataset, younger patients with SVD shared common vascular RF with older patients. In the group aged ≤55, better functional and cognitive status and less severe MRI changes were noted. However, a substantial number of younger SVD patients presenting with TIA or ischemic stroke had various deficits.

Increased Frequency of Pathologic Findings on Transcranial B-Mode Parenchymal Sonography in Patients with Sporadic Amyotrophic Lateral Sclerosis

Although amyotrophic lateral sclerosis (ALS) is characterized by involvement of motor neurons in the motor cortex, brainstem and spinal cord, there is accumulating evidence that it is a multisystem degenerative disease, with dysfunction of the striatonigral dopaminergic system as well. Transcranial B-mode sonography of the parenchyma enables depiction of the differing tissue echogenicity of midbrain and basal ganglia structures in various movement disorders. Transcranial B-mode sonography was performed in the standard manner in 101 patients with sporadic newly diagnosed ALS and 60 age- and gender-matched controls. Increased frequencies of pathologic substantia nigra hyper-echogenicity (p = 0.027), interrupted brainstem raphe (p = 0.003) and increased third ventricle diameter (p < 0.0001) were detected in ALS patients as compared with healthy controls. Only four ALS patients exhibited some features of parkinsonism. Pathologic findings on transcranial B-mode sonography of parenchyma did not correlate with clinical presentation, functional status or disease subtype. Our study provides additional evidence of multisystem involvement in ALS patients, particularly in subcortical areas.

Long-term predictors of remission in patients treated for medication-overuse headache at a specialized headache center: A prospective cohort study

Objective To evaluate long-term predictors of remission in patients with medication-overuse headache (MOH) by prospective cohort study. Background Knowledge regarding long-term predictors of MOH outcome is limited. Methods Two hundred and forty MOH patients recruited from 2000 to 2005 were included in a one-year follow-up study and then subsequently followed until 31 December 2013. The median follow-up was three years (interquartile range, three years). Predictive values of selected variables were assessed by the Cox proportional hazard regression model. Results At the end of follow-up, 102 (42.5%) patients were in remission. The most important predictors of remission were lower number of headache days per month before the one-year follow-up (HR-hazard ratio = 0.936, 95% confidence interval (CI) 0.884–0.990, p = 0.021) and efficient initial drug withdrawal (HR = 0.136, 95% CI 0.042–0.444, p = 0.001). Refractory MOH was observed in seven (2.9%) and MOH relapse in 131 patients (54.6%). Conclusions Outcome at the one-year follow-up is a reliable predictor of MOH long-term remission.