Serotonin syndrome is a potentially life-threatening drug-induced toxidrome associated with increased serotonergic activity in both the peripheral and central nervous systems. It is characterized by a broad spectrum of clinical findings, which include mental state changes, autonomic instability, and hyperexcitability of CNS with neuromuscular abnormalities. Serotonin syndrome can arise usually by simultaneous administrations of 2 or more serotoninergic drugs, the combination including monoamine oxidase inhibitors is especially dangerous, but occurrence in monotherapy is also possible. This review describes pathophysiology and clinical manifestation of this syndrome and the drugs at risk, with a particular focus on drugs used in the treatment of chronic pain.

Lennox-Gastaut syndrome (LGS) represents a syndrome whose characteristics have recently been reviewed by the International League Against Epilepsy (ILAE). LGS is characterized not only by its development in childhood, but also by significant pharmacoresistance that greatly affects the quality of life of patients and their family members. The presence of specific seizures, including tonic seizures and at least one other type of seizure (atypical absences, atonic or myoclonic seizures, tonic-clonic seizures, focal impaired awareness seizures, non-convulsive status epilepticus, and epileptic spasms), is one of the key diagnostic features. An EEG investigation then reveals specific findings that are typical for this syndrome. In our article, we will focus in more detail on all these clinical and neurophysiological characteristics, their variability, and diagnostic specificities. We will also deal with the issue of diagnosing adult patients with LGS, which is an area that often poses a challenge in the clinical practice. In this context, we will introduce a specific screening tool - the REST-LGS questionnaire (Refractory Epilepsy Screening Tool for Lennox-Gastaut syndrome). In conclusion, we will highlight current treatment options that are suitable for patients with LGS, as well as mention novel and innovative therapeutic approaches, such as cannabidiol (CBD) or fenfluramine. The aim of this article is to increase awareness of the neurological community regarding the importance of accurately diagnosing LGS. The article is primarily focused on neurologists in charge of treating adult patients, rather than on paediatric neurologists who - in the vast majority of cases - are well acquainted with this entity.

Dravet syndrome (DS) is predominantly caused by a mutation in the sodium channel subunit (SCN1A). It begins in childhood but is a lifelong condition. In this article, we focus on the characteristics of adult patients with DS. From an epileptological perspective, the occurrence of epileptic seizures is crucial. In adulthood, there are mainly clonic seizures (generalized tonic-clonic seizures, tonic seizures or focal seizures to bilateral tonic-clonic seizures). They can be provoked by increased body temperature or by emotions. However, parents/caregivers of patients with DS often report other manifestations of the disease as very significant, often more limiting than the seizures themselves. These primarily include cognitive difficulties, learning disorders, behavioral issues, motor problems (crouch gait, parkinsonism, ataxia, abnormal postures while standing and walking), sleep disorders, and cardiac abnormalities. There is also a high risk of sudden unexpected death in epilepsy (SUDEP) in DS. The second part of the article is dedicated to therapy. Currently, there are recommendations for patients with DS. The first line of therapy includes valproate and clobazam. The second line involves specific drugs indicated for this syndrome, specifically stiripentol (Diacomit), fenfluramine (Fintepla), and cannabidiol (CBD, available in the Czech Republic as Epidyolex).

Takotsubo syndrome is defined as a syndrome of acute heart failure that is very often manifested clinically as acute coronary syndrome. Transient left ventricular dysfunction is a characteristic clinical feature. Acute neurological diseases are relatively frequently the triggering factors; thus, it is assumed that it is the central nervous system that plays a significant role in the pathophysiology. The review article aims to provide basic information on Takotsubo syndrome with a focus on the very likely pathophysiological mechanisms in the brain-heart axis.

Central Post-Stroke Pain (CPSP) is defined as a painful condition after a central stroke with a proven lesion in the central nervous system (CNS). Clinical signs appear as early as 1-3 months after the stroke, but in most affected patients it is up to 6 months. Major clinical manifestations include tension headache, pain associated with muscle spasticity and pain shoulder syndrome with possible development of complex regional pain syndrome (CRPS). The treatment combines the use of analgesics (amitriptyline, carbamazepine, gabapentinoids), physiotherapy and psychotherapeutic procedures.

Neck-tongue syndrome is a rarely occurring disease which is included and defined in the International Classification of Headache Disorders (ICHD-3). The clinical manifestations of neck-tongue syndrome (NTS) are sudden-onset headache at the back of the neck and sensory-motor signs due to impairment of tongue innervation, occurring as a result of vigorous head movement. In the clinical practice, the diagnosis is made by exclusion, that is after ruling out more frequent and potentially more serious conditions, particularly vascular pathologies (pre/intracerebral artery dissection and subarachnoid haemorrhage). The treatment predominantly involves nonpharmacological approaches represented by physiotherapy of the suboccipital and cervical regions. A case report is presented of a 53-year-old female patient admitted for acute-onset dysarthria and severe headache, occurring as a result of sneezing hard.

Creutzfeldt-Jakob disease (CJD) is an irreversibly fatal disease caused by a pathologically conformed prion protein. Although it can also occur at a younger age, it is found in a surprisingly high percentage in all age groups in comorbidity with other neuropathological entities. In many cases, the comorbidities can change the typical clinical symptomatology of patients and thus significantly complicate clinical diagnosis. We present the case report of a 75-year-old female patient, in whom definitive neuropathological examination revealed a combination of sporadic Creutzfeldt-Jakob disease, fully developed dementia with Lewy bodies, fully developed Alzheimer's disease, frontotemporal lobar degeneration with predominance of features of age-related astrogliopathy, and relatively advanced vascular dementia. Although comorbidities of other neurodegenerative diseases are common in CJD, in this case it was a very unusual combination of multiple developed neurodegenerations.

Fasciculations are one of the uncommon but potentially serious neurological symptoms and can occur in a variety of neurological and other diseases or conditions. They can arise for many different reasons: from metabolic-endocrine (calcium and magnesium metabolism disorder, hyperthyroidism), through drug side effects (succinylcholine, depolarizing muscle relaxants, acetylcholin inhibitors, salbutamol) to serious neurological diseases (ALS/MND, neuropathy, root syndromes, borreliosis). They are often the result of increased neuromuscular irritability during excessive use of stimulants (caffeine, tein) or increased anxiety, or after sudden physical overload. If the condition is persistent and we are unable to find the cause of the fasciculations, then the finding can be described as a benign fasciculation syndrome that has a good prognosis.

Susac's syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, arterial retinal occlusion, and neurosensory hearing loss based on microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by a typical radiological finding on MRI, which, together with clinical symptoms, may allow diagnosis. Retinal artery occlusion is best evaluated by fluorescein angiography, which may exhibit typical multifocal fluorescence. SS is an autoimmune endothelopathy that requires long-term treatment with corticosteroids, immunosuppressants, or even monoclonal antibodies, in acute cases intravenous immunoglobulin or plasmapheresis. Therapy leads to significant clinical and radiological improvement. Early diagnosis and treatment is important for delaying disease progression and preventing permanent disability. The article is illustrated by a case report of a woman with typical SS manifestations.

Choroba či syndrom moyamoya by měl být zvažován v rámci diferenciální diagnostiky jako příčina subarachnoidálního krvácení, pokud není jako jeho potenciální zdroj nalezena jiná zřejmá cévní patologie. Moyamoya disease is a chronic cerebrovascular disease characterized by progressive stenosis and/or occlusion of the intracranial internal carotid and its proximal branches with a created abnormal collateral network of small arteries. If the vascular occlusion is not bilateral and the patient has been diagnosed with a specific underlying illness, we refer to this condition as Moyamoya syndrome. The presentation of Moyamoya disease or syndrome in the form of a non-aneurysmal subarachnoid hemorrhage is rare. The 62-year-old woman was admitted for 24 hours lasting pain in the area of the neck, forehead, and temples. She was somnolent, vomited repeatedly, and had double vision. The brain computed tomography discovered subarachnoid hemorrhage. The brain CT angiography revealed an occlusion of the left middle cerebral artery and the digital subtraction angiography, moreover, showed a network of tiny tortuous moyamoya vessels in the left middle cerebral artery territory. The brain perfusion was not impaired. The patient was treated with corticosteroids and anti-platelet drugs. A diagnosis of Moyamoya syndrome was determined. She was dismissed without neurological deficit or subjective problems. After six years, the patient`s general condition deteriorated because of sepsis, and even after getting maximum antibiotic therapy, she died. Moyamoya disease or syndrome should be considered in the differential diagnosis as the cause of subarachnoid hemorrhage in the absence of other clear vascular pathology as its potential source.

In the following review article, we introduce fenfluramine - an "old-new" drug, used in the past for the treatment of obesity and now relatively newly registered in the European Union, the USA and the UK as an orphan drug for the therapy of epileptic seizures associated with Dravet and Lennox-Gastaut syndrome, i.e. with severe and generally ultrarefractory epileptic encephalopathies. The mechanism of action of fenfluramine is based on interaction with serotoninergic and sigma-1 receptors, which, in addition to reducing seizure activity, promises to patients cognitive, emotional, and behavioral benefits. Efficacy and a safety profile have been proved in four randomized phase III trials and subsequently confirmed in two follow-up open-label studies.

Autoimmune encephalitides (AIE) are autoimmune diseases of the central nervous system with predominant involvement of the cerebral cortex. The prevalence of AIE is comparable to that of encephalitis of infectious etiology. They can occur as paraneoplastic syndromes (in which the abnormal immune response is triggered by the presence of a peripheral tumor) or as non-paraneoplastic syndromes (where a combination of a viral trigger and an innate disposition seems to play a role). The most common AIEs include encephalitis with antibodies to glutamate N-methyl-D-aspartate receptors (NMDAR encephalitis), limbic encephalitis with antibodies to leucine-rich glioma inactivated protein 1 (LGI1 encephalitis) and syndromes formerly referred to as "classic paraneoplastic" - in today's terminology "high-risk phenotypes". These include limbic encephalitis with anti-Hu and anti-CV2 positivity, rapidly progressive cerebellar syndrome (usually associated with anti-Yo antibodies) and stiff-person syndrome (including its variants). In recent years, several new phenotypes have been described that deserve attention, such as anti-GFAP syndrome, anti-GABAA receptor encephalitis (relevant in the context of new-onset refractory status epilepticus - NORSE) and syndrome with anti-IgLON5 antibodies. This article provides a basic overview of current information regarding AIE.

Tourette syndrome (TS) is one of the most common neuropsychiatric disorders of childhood. The usual age of symptom onset is between the ages of 5 and 6 years, and the vast majority of cases emerge by age 12, up to four times more often in boys than in girls. The course of the disease characteristically varies over time, with exacerbations, remissions, and changes in the tic pattern. In adolescence, the tics usually diminish and marked late exacerbations are rare. However, since 2020, the number of new cases of tic disorder has increased sharply in adolescent girls. Their manifestations are incongruent with the classical presentation of TS and are suggestive of a functional (psychogenic) origin. They show strikingly similar features to videos circulated on social media. We present the typical manifestations of the disease in several of our patients. Using the concept of mass sociogenic illness, we describe the role of social media in its spread and draw parallels with historical mass psychiatric phenomena.

Opsoclonus-myoclonus syndrome is a rare and heterogeneous disorder of children and adults. Main clinical features, as the name of the syndrome suggests, are opsoclonus and myoclonus of trunk and limbs. Next features are ataxia, dysarthria, behavioral and sleep disturbance. In this case report we will look into OMS in adults, his diagnostics, finding out an etiology a his treatment. The treatment is imunomodulating, sympthomatic, in case of paraneoplastic etiology of course the treatment of primary neoplasia. Physiotherapy, logopedia, ergotherapy and psychotherapy play an important part in the treatment too.

We report a case of a patient with clinical symptoms resembling cauda equina syndrome, however, with a negative finding on imaging studies. Due to severe pain and progressive limb weakness, a series of examinations were performed based on which dermatomyositis was suspected. Despite intensive care and causal treatment with corticoids, the patient expired. Histopathological changes characteristic for polyarteritis nodosa were observed postmortem. Polyarteritis nodosa is a rare disease which may be neglected in the differential diagnosis for inflammatory myopathies.

GBS is an autoimmune neuropathy and is the most frequent cause of acutely deve­loped flaccid paresis. Infect or an immune impuls precede in 70 %. Molecular mimicry participates in pathogenesis. The illness developes till 4 weeks, but commonly during 2 weeks. In typical cases the GBS begins with hypesthesia and weakness of distal parts of lower etremities and then spreads in ascedent direction. Facial nerve is affected in 50 %. Paresis of ventilatory muscles may be a cause of intubation - in 30 %. Mortality undulates between 3 % and 10 %. After 6 months there are 60-80 % of patients able to walk unsupported. The relapses occure in 2-5 %. Electrophysiological investigation is very important and it defines demyelinating or axonal type of lesion. Clinical findings can be devided in generalized forms, focal forms and Miller-Fisher syndrome.

Dravet syndrome (DS) is ranked among severe epileptic syndromes with occurence in the first year of life in normal children. It can be diagnosed according to the clinical course, genetics can be very helpful by assessing the mutation in SCN1A gene, which is responsible for 70–80 % of cases with DS. Other mutations were identified more rarely (SCN2A, SCN3A, SCN7A, SCN8A a SCN9A, GABARG2, SCN1B and PCDH19). Dravet syndrom in adulthood is characterised by cognitive and behavioral changes in patients with various rate of mental retardation, language deficit and cerebellar symptomatic. The course of epilepsy is milder, the rate of seizure freedom is still low. Patients often suffer from nocturnal partial complex seizures with secondary generalisation, often with frontal origin. Present possibilities of genetic confirmation of DS are very important from the therapeutical point of view. This allows to improve the prognosis of the disease and the quality of life of patients with DS.

Stiff person syndrome (SPS) is a rare autoimmune disease characterized by progressive fluctuating muscle hypertonia and superimposed painful muscle spasms, resulting in a gait disorder. Its variant – stiff limb syndrome (SLS) – particularly affects limb muscles, with axial muscles being affected substantially less. The disease may respond favourably to immunosuppressive therapy. The video case report presents a female patient examined repeatedly for transient lower limb stiffness. The patient has been suspected to have a functional movement disorder. The disease had progressed gradually and led to loss of self-sufficiency and becoming bedridden. The patient was subsequently found to be positive for anti-glutamic acid decarboxylase antibodies (anti-GAD-Ab), which is characteristic for SPS/SLS. The introduction of immunosuppressive therapy resulted in a significant improvement in the clinical presentation and quality of life of the patient. The video case report of this patient with SLS primarily aims at highlighting the importance of comprehensive patient evaluation and the need to be aware of rare neurological entities.

Dravet syndrome is a developmental and epileptic encephalopathy starting in infancy and its main features are drug -resistant epilepsy and several co-morbidities. Seizures are typically provoked by increased temperature. The treatment of Dravet syndrome is challenging. The first antiseizure drug should be valproic acid, while clobazam, stiripentol, fenfluramine, canabidiol or topiramate are usually added later. All the patients must have rescue medication for home management of seizures. Sodium channel blockers should not be used for chronic treatment, but phenytoin can be administered to stop status epilepticus. Non-pharmacological treatment of co-morbidities should be addressed as well.

The association of headaches and multiple sclerosis (MS) has been found in the last years in more studies. Most of them are of migrainous type. The prevalence of migraine in MS patients is significantly higher (46%) than in general population (10-15%). This association is considered in clinical practice usually as a comorbidity of two independent diseases. Some facts however indicate, that headaches, especially of migrainous type, could be also the symptom of MS. Then they would belong to the secondary headaches. The headache occurs with increased frequency especially in the initial stage of MS. A prospective multicenter study has revealed the occurrence of headache in 78% of patients with clinically isolated syndrome (CIS) or early MS. Most often patients suffered from throbbing and pulsating headaches of migrainous type. The headache is also in half of cases the cause of brain MRI (magnetic resonance imaging) performance in radiologically isolated syndrome(RIS). This high frequency raises the question, whether it is really a comorbidity of two independent diseases or whether the headache could be also a primary syndrome of MS. Clinically and therapeutically this is very important, as presence of headache alone would allow the classification as CIS instead of RIS and to treat the patients accordingly with immunomodulatory therapy. In addition nowadays the question is being discussed, whether to treat already in the stage of RIS.

Cauda equina syndrome is complex neurological syndrome most often caused by extradural compresssion of the lumbar and sacral spinal roots due to acute medial disc herniation. Typical clinical symptoms are radicular pain, saddle hypoesthesia, anesthesia or paraesthesia, sphincter dysfunction, weakness of the lower extremities and impaired low reflexes. Cauda equina syndrome could manifest incompletely and asymmetric disability is frequent. Multiple sclerosis (MS) is progressive autoimmune chronic inflammatory disease of the central nervous system triggered by environmental factors in a genetically predisposed individual. MS attack can affect all parts of the central nervous system. Voiding disorders are the only one symptom at the onset of the disease in 2 % of patients while they are found in 50–97 % of patients in a fully developed MS. This article points to possible issues in the differential diagnosis of these two diseases.

In this case report we present the diagnostic process and treatment of a patient with severe chronic polyneuropathy (lasting around 3months), lymfadenopathy. hepatosplenomegaly, hypertrichosis and other symptoms. The patient´s condition required consultations with a neurologist, a hematologist, an ophtalmologist and also with a rehabilitation specialist and infectologists. The diagnose classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was considered in the beginning due to relevant clinical manifestations. Intravenous immunoglobulins did not lead to significant clinical improvement of the polyneuropathy. Afterwards, it was found that our patient suffers from POEMS syndrome - a rare diagnosis where the hematological examination is crucial. This acronym means Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes. Our patient did exhibit all five of those symptoms. In the end, the neurological manifestations improved significantly after specialized treatment (including autologous transplantation of stem cells). The patient´s quality of life improved, i.e. disability disappeared almost completely thanks to multidisciplinary care. We discuss different aspects of the diagnostic process in POEMS syndrome which included hospitalization in our neurology clinic. The treatment plan is also presented here. POEMS syndrome is a rare and complex disease where the neurologist plays an important role in the differential diagnostic clarification.

The mainstay of treatment of all subtypes of idiopathic inflammatory myopathies, with the exception of inclusion body myositis, is pharmacotherapy with immunosuppressive and immunomodulatory agents. Different subtypes of the disease require different therapeutic approaches. Detection of myositis-specific autoantibodies, which are associated with a characteristic phenotype and response to therapy, plays an important role in deciding the choice of drug. It is always necessary to distinguish between true disease activity and damage, which cannot be influenced by pharmacotherapy. Given the increased incidence of malignancies, cancer screening is an important part of care. A number of clinical trials with new drugs have gradually expanded the therapeutic options in recent years. In particular, the results of T cell therapy with chimeric antigen receptor have attracted a lot of attention; single course of treatment has a potential to induce drug-free remission.

Congenital and developmental disorders of the spine and spinal cord in the lumbosacral region in childhood are affections divided into two main groups - spina bifida aperta and spina bifida occulta. These dysrapisms in many cases significantly affect the motor and sensitive functions of the lower limbs, deterioration of urine and faeces. We present the most frequent clinical units, their diagnosis and treatment.

Anterior choroidal artery syndrome is a rare clinical syndrome consisting of a triad of contralateral severe hemiparesis/hemiplegia,hemiparesthesia and homonymous hemianopia, typically as a result of a cerebral infarction in the anterior choroidal artery territory.A 72-year-old woman was admitted for a left-sided hemiplegia, hemianesthesia, facial palsy, neglect syndrome/anosognosia andleft homonymous hemianopia. Control magnetic resonance has showed acute ischemic changes in the posterior limb of internalcapsule corresponding to the territory of the right anterior choroidal artery.

A fixed combination of paracetamol and tramadol per tablet enhances analgesic efficacy and reduces side effects of the individual components. This expands the portfolio of analgesics to treat acute and chronic pain, especially in elderly and at-risk patients. The article concludes by describing the practical use of analgesics in the treatment of back pain.

Stiff limb syndrom is characterized by muscle stiffness and episodic painful spasms of one or two extremities. Pathophysiological basis of this syndrome is a disturbance of spinal motoneurone inhibition. For a correct diagnosis are of upmost importance – electromyographic investigation with continuous motor units activity. Finding of antibodies against glutamic acid decarboxylase in blood and cerebrospinal fluid is of supportive value. For therapy are used – drugs increasing gabaergic inhibition (diazepam, baclofene), immunosuppression (methylprednisolone i. v., immunoglobulin i. v.) or therapeutic plasma exchange. The authors present a case report of 65-year old woman with a severe form of stiff limb syndrome with asymmetric stiffness of both lower extremities, difficult ambulation, typical findings and a very good response to therapy (methylprednisolone i. v.)

We present a case report of a 61-year-old man with an acute onset of epileptic seizures, encephalopathy, delirium, peripheral nerve hyperexcitability syndrome and dysautonomy with indentified anti-Caspr2 antibodies whose clinical status has promptly improved after a high dose of corticosteroids. Subsequently a short review of anti-Caspr2 pathophysiology, spectrum of clinical manifestation with emphasis put on a typical Morvan syndrome, diagnostics and treatment of these conditions is presented.

Authors would like to present a case of an uncommon entrapment syndrome of the anterior antebrachial interosseal nerve ("The Kiloh-Nevin syndrome). Patient was first examined by an ambulatory neurologist. EMG verified minimal neuropathic finding of the median nerve and clinical presentation first lead to the diagnosis of carpal tunnel syndrome. After a neurosurgical consult on the authors departement we found the corresponding clinical presentation misleading including the EMG presentation. Newly recommended EMG uncovered anterior antebrachial interosseal nerve neuropathy (Kiloh-Nevin syndrome). After a failed conservative therapy, patient underwent a surgical intervention with a good outcome.