Reversible splenial lesion syndrome in patient with acute motor and sensory axonal neuropathy.

Even though the classical clinical concept supports the clear difference between diseases affecting the central and peripheral nervous systems, this difference is becoming less rigid. Here, we report the case of a 50-year-old male patient who presented with acroparaesthesia, headache, and flaccid tetraparesis after febrile diarrhea. Nerve conduction studies indicated action potentials with low amplitudes, which are typical in acute motor and sensory axonal neuropathy. Magnetic resonance revealed a round lesion in the splenium consistent with a diagnosis of reversible splenial lesion syndrome. A polyclonal antiganglioside antibody response was detected. The patient was successfully treated with intravenous immunoglobulins. The coexistence of reversible splenial lesion syndrome and acute motor and sensory axonal neuropathy has not been described in the literature so far. We discuss our diagnostic dilemmas and the possible role of antiganglioside antibodies in the occurrence of simultaneous lesions of the central and peripheral nervous systems.

Frank's Sign: A Link Between Dermatovenerology, Cardiac Pathology, and Neurology.

Dear Editor, Although some of my colleagues may find this surprising, as a neurologist, I have noticed many connections between dermatology and neurology. Neurological and dermatological signs and symptoms are common in many clinical entities, especially in the so-called phakomatoses or neurocutaneous syndromes (Von Recklinghausen's disease type 1 and 2, Bourneville-Pringle syndrome, Sturge-Weber syndrome, Von Hippel-Lindau syndrome, Louis-Bar syndrome) (1). The terms "neurodermatitis" and "neurodermatology" also confirm the above. Inspection is the basis of every clinical examination and an integral part of both dermatological and neurological propaedeutics. Therefore, I would like to remind your readers of Frank's sign, another link between dermatology and neurology. Frank's sign is a diagonal earlobe crease (DELC) that extends backwards from the tragus at a 45-degree angle across the lobule to the auricular edge of the ear (Figure 1). It has been described as a dermatological marker for atherosclerosis. Frank's sign is named after Dr. Sanders T. Frank, who observed this crease in 20 patients with coronary artery disease and published his findings in The New England Journal of Medicine in 1973 (2). Although this sign has been known for more than 50 years, it is still not routinely employed in clinical practice. Histopathological examination of DELC-positive earlobes revealed myoelastofibrosis in the arterial vessel at the base of the earlobe, indicating that DELC is not a coincidental finding but is directly related to atherosclerosis (3). Following the finding of DELC in patients with coronary artery disease, numerous studies have confirmed the presence of DELC in peripheral vascular disease as well as cerebrovascular disease. I encountered the description of this sign as a student in the textbook of Internal Medicine in 1991 (4). This sign was also the subject of research by Croatian authors. In 1998, Miric et al. found that a positive Frank's sign carried a higher risk of heart attack (5,6). In 2008, Glavic et al. found a statistically significant association between Frank's sign and an increase in intima media thickness (IMT) of the common carotid artery as a surrogate marker of atherosclerosis, thus confirming the hypothesis that Frank's sign is an uncontrollable risk factor for cerebrovascular disease (such as gender or age) (7). In clinical practice, earlobe inspection should be considered an integral part of the physical examination. In the case of a positive Frank's sign, a color Doppler ultrasound examination of the neck arteries and a cardiologist's examination are recommended. The determination of Frank's sign can be used as a method of primary prevention for cardiovascular and cerebrovascular diseases.

Complex regional pain syndrome type I after diphtheria-tetanus (Di-Te) vaccination.

Complex regional pain syndrome type I (CRPS I) is a disorder of one or more extremities characterized by pain, abnormal sensitivity (allodynia), swelling, limited range of motion, vasomotor instability, fatigue and emotional distress. The symptoms may be aggravated by even minor activity or weather change. It is usually provoked by injury, surgery or injection but in a small proportion of patients CRPS I develops without a clear causative event. There are several literature reports on CRPS after rubella and hepatitis B vaccination. We present a case of CRPS I affecting the left arm after diphtheria and tetanus (Di-Te) vaccination in the left deltoid muscle in a young girl having experienced profound emotional stress before the vaccination procedure. History data on previous minor trauma at the site of vaccination or emotional stress may necessitate temporary vaccination delay due to their proneness to impaired local or systemic immune response and CRPS as a complication of vaccination. If a child or an adult has prominent swelling and severe pain after vaccination, the diagnosis of CRPS I should be considered and if confirmed, the multidisciplinary treatment should start as soon as possible.

[Stiff person syndrome (Moersch-Woltman)]. / Sindrom ukocene osobe (Moersch-Woltman).

The prime goal of this paper is to offer an overview of main scientific points in epidemiology, genetics, pathogenesis, clinical course and therapeutic strategies in stiff person syndrome (SPS). This syndrome is characterized by progressive muscle rigidity and painful muscle spasms. Three major forms of SPS are described, according to the pathophysiologic basis, autoimmune, paraneoplastic and idiopathic SPS. In autoimmune form of SPS the antibodies are specific for an enzyme (glutamic acid decarboxylase, GAD). If the paraneoplastic form takes place, the antibodies may be specific for presynaptic (amphyphysin) or the postsynaptic protein (gephyrin). The SPS diagnosis should be based on clinical, laboratory and electromyoneurographic criteria, according to Gordon and Lorish. The therapeutic approaches are focused on symptomatic therapy managing the muscle spasm and on possible immunomodulatory procedures to attenuate an autoimmune reaction. Two cases of SPS are reported in the Republic Croatia since 2005. Although it is a rare medical condition, SPS is of clinical importance, especially because it may be the first sign of an underlying undiagnosed malignant disease or if the anesthesia is necessary in SPS patient.

Stiff-person syndrome first manifesting in pregnancy.

Stiff-person syndrome (SPS) is a rare neurological disorder characterised by progressive stiffness and painful muscle spasms. We present a case of the autoimmune form of glutamate decarboxylase-positive SPS that initially manifested in pregnancy. The diagnosis was made based on clinical, laboratory and electromyoneurographic criteria. The patient was administered low doses of diazepam and baclofen. Considering the clinical picture of SPS patients, caesarean section is the method of choice for pregnancy termination.

Neuroanatomical basis of Sandifer's syndrome: a new vagal reflex?

Sandifer's syndrome is a gastrointestinal disorder with neurological features. It is characterized by reflex torticollis following deglutition in patients with gastroesophageal reflux and/or hiatal hernia. The authors believe that neurological manifestations of the syndrome are the consequence of vagal reflex with the reflex center in nucleus tractus solitarii (NTS). Three models for the neuroanatomical basis of the hypothetic reflex arc are presented. In the first one the hypothetic reflex arc is based on the classic hypothesis of two components nervus accessorius (n.XI) - radix cranialis (RC) and radix spinalis (RS) The nervous impulses are transmitted by nervus vagus (n.X) general visceral afferent (GVA) fibers to NTS situated in medulla oblongata, then by interneuronal connections on nucleus ambiguus (NA) and nucleus dorsalis nervi vagi (NDX). Special visceral efferent fibers (SVE) impulses from NA are in part transferred to n.XI ramus externus (RE) (carrying the majority of general somatic efferent (GSE) fibers) via hypothetic anastomoses in the region of foramen jugulare. This leads to contraction of trapezius and sternocleidomastoideus muscles, and the occurrence of intermittent torticollis. In the second suggested neuroanatomical model the hypothetic reflex arc is organized in the absence of n.XI RC, the efferent part of the reflex arc continues as NA, which is motor nucleus of nervus glossopharyngeus (n.IX) and n.X in this case while distal roots of n.XI that appear at the level of the olivary nucleus lower edge represent n.X roots. In the third presented model the hypothetic reflex arc includes no jugular transfer and could be realized via interneuronal connections directly from NTS to the spinal motoneurons within nucleus radicis spinalis nervi accessorii (NRS n.XI) or from NA to NRS n.XI. The afferent segment of the postulated reflex arc in all three models is mediated via n.X. We conclude that Sandifer's syndrome is a clinical manifestation of another vagal reflex that could be termed a "vagocervical" or "esophagocervical" reflex, based on the neuroanatomical hypotheses elaborated in this paper.

[Restless legs syndrome (Wittmaack-Ekbom) "a most common disorder that you never heard of"]. / Sindrom nemirnih nogu (Wittmaack-Ekbom) "tako cest, a nedovoljno poznat poremecaj".

The aim of this article is to provide a survey of scientifically proven knowledge about the Restless Legs Syndrome (RLS). The RLS is a neurological condition, a disorder of movement with the prevalence amounting from 2 to 15%. Primary RLS types are often familial, while the secondary RLS types are sporadic, most often caused by iron deficiency, uremia, pregnancy, polyneuropathy, the use of certain medications. The diagnosis is based on international diagnostic criteria, while the treatment is based on the use of dopaminergic agents, opioids, anticonvulsants and benzodiazepines. Doctors specialized in different clinical fields encounter patients with RLS, but they rarely diagnose it due to being uninformed. The adoption of these knowledges would ensure conducting epidemiological studies in the future, which would result in demonstrating real data on the spread of this illness within the population of Croatia.