Nadezda Sternic

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.

Clinical manifestations, diagnostic criteria and therapy of Hashimoto's encephalopathy: report of two cases.

Hashimotos encephalopathy (HE) is a rare, still not well understood, autoimmune disease with neurological and psychiatric manifestations. and elevated titers of antithyroid antibodies in serum and cerebrospinal fluid (CSF) as a hallmark of the disease. Patients are mostly women. Current diagnostic criteria include corticosteroide responsiveness, but it is the case in only 50% of patients with HE. In steroid non-responders other immunomodulatory therapies or plasmapheresis could be applied. Disease course can be acute, subacute, chronic or relapsing-remitting. Two distinct forms emerged from the reported cases: a vasculitic type characterized by multiple relapsing-remitting stroke-like episodes and mild cognitive impairment and a diffuse progressive type characterized by dementia and psychiatric symptoms. Both forms may be accompanied by depressed level of consciousness (stupor or coma), tremor, seizures, or myoclonus. We present two patients with two distinct forms of HE who had different clinical manifestations and response to therapy.

Calcitonin gene-related peptide levels in saliva of patients with burning mouth syndrome

Burning mouth syndrome (BMS) is an intraoral burning sensation for which no medical or dental cause can be found. Recent studies suggest that primary neuropathic dysfunction might be involved in the pathogenesis of BMS. Calcitonin gene-related peptide (CGRP) plays an important role in the development of pain and serves as a biological marker of trigeminovascular activation. The aim of this study was to determine the levels of CGRP in the saliva of BMS patients and estimate the trigeminovascular activation in BMS. CGRP levels were measured, by RIA method in 78 BMS patients and 16 healthy subjects. The levels of CGRP were non-significantly decreased in BMS patients in comparison to healthy subjects. These results suggest that trigeminal nerve degeneration may be the underlying cause of BMS.

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.

Underestimated phenomena: Higher cortical dysfunctions during migraine aura

Introduction Aura occurs in 20–30% of patients with migraine. Some descriptions of aura go far beyond the most frequent visual and sensory symptoms, suggesting the involvement of different cortical areas. The aim of this prospective study was to evaluate the frequency and types of disorders of higher cortical functions (HCF) that occur during visual and/or sensory aura. Methods We interviewed 60 patients with visual and/or sensory aura about HCF disorders of praxia, gnosia, memory, and speech, during aura. Patients were divided into two groups, with and without HCF disorders, and were compared in terms of demographic data and aura characteristics. Results From all 60 patients, 65% reported at least one HCF disorder during aura. The patients with HCF disorders had longer-lasting auras (28.51 ± 16.39 vs. 19.76 ± 11.23, p = 0.016). The most common HCF disorders were motor dysphasia (82.05%) and dysnomia (30.74%). Motor dysphasia was more often reported by patients with visual as well as sensory aura (p = 0.002). The number of HCF disorders correlated with the aura duration (p = 0.003). Conclusion According to our results, HCF disorders during aura occur more often than previously thought. The aura duration has some influence on the HCF disorders.

Multiple Sclerosis as the Cause of Sudden ‘Pontine’ Deafness

Sudden deafness is rarely ascribed to multiple sclerosis (MS). Sudden deafness and tinnitus were the initial symptoms of MS for the two patients described in this paper. A sensorineural hearing loss was present in one ear in both patients. Brainstem responses (BSRs) showed only the first three waves for the first patient, and only wave I for the second. Magnetic resonance imaging showed focuses of demyelination in the pons (case 1) and on the borderline between medulla and pons (case 2). The placement of plaques and the involvement of the BSR-generating acoustic afferent pathways are discussed. The hearing level measured by tonal audiometry recovered after 1 month in both patients and remained stable during 1 year. BSRs remained pathological after 1 month as well as after 1 year. Sudden hearing loss and tinnitus might be the initial symptoms of MS.

Adult-onset autosomal dominant leukodystrophy without early autonomic dysfunctions linked to lamin B1 duplication: a phenotypic variant

The early presentation of autonomic dysfunctions at the disease onset has been considered the mandatory clinical feature in adult-onset autosomal dominant leukodystrophy, which is a rarely recognised leukodystrophy caused by duplication of the lamin B1 gene. We report the first family with adult-onset autosomal dominant leukodystrophy and lamin B1 duplication, without the distinguishing early-appearing autonomic dysfunctions. Subjects from three consecutive generations of a multi-generational Serbian family affected by adult-onset autosomal dominant leukodystrophy underwent clinical, biochemical, neurophysiological, neuroradiological, and genetic studies. The patients atypically exhibited late autonomic dysfunctions commencing at the disease end-stages in some. Genetic findings of lamin B1 duplication verified adult-onset autosomal dominant leukodystrophy, which was supported also by neuroimaging studies. Exclusively, proton magnetic spectroscopy of the brain revealed a possibility of neuro-axonal damage in the white matter lesions, while magnetic resonance imaging of the spinal cord excluded spinal myelin affection as a required finding in this leukodystrophy. The detection of lamin B1 duplication, even when autonomic dysfunctions do not precede the other symptoms of the disease, proves for the first time that lamin B1-duplicated adult-onset autosomal dominant leukodystrophy may have a phenotypic variant with delayed autonomic dysfunctions. Prior to this report, such a phenotype had been speculated to represent an entity different from lamin B1-duplicated leukodystrophy. Hereby we confirm the underlying role of lamin B1 duplication, regardless of the autonomic malfunction onset in this disorder. It is the only report on adult-onset autosomal dominant leukodystrophy from Southeastern Europe.

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.

Clozapine in hemiballismus: report of two cases.

Hemiballism is a relatively rare hyperkinetic disorder; treatment is based mainly on neuroleptics and drugs that decrease release of dopamine. We report the cases of two patients with hemiballism. After a period of 1 month of nonresponsiveness to haloperidol, amelioration of ballistic movements was observed only a few days after the initiation of clozapine therapy (50 mg/day). Our report suggests that clozapine may be a valuable alternative for patients with hemiballism.

Migraine-Like Accompanying Features in Patients With Cluster Headache. How Important Are They?

According to the International Classification of Headache Disorders diagnostic criteria, the differences between migraine and cluster headache (CH) are clear. In addition to headache attack duration and pain characteristics, the symptoms accompanying headache represent the key features in a differential diagnosis of these 2 primary headache disorders. Just a few studies of patients with CH exist examining the presence of nausea, vomiting, photophobia, phonophobia, and aura, the features commonly accompanying migraine headache.