Clinical Neuroscience

Atypical type of Hirayama disease: Onset of proximal upper extremity

AYAS Özözen Zeynep1, ASIL Kıyasettin2

JULY 30, 2018

Clinical Neuroscience - 2018;71(07-08)


Hirayama disease is a rare, benign motor neuron disease. It has been proposed that the dura mater’s posterior wall lacks sufficient elasticity in the lower cervical region and this causes the tense dura part to displace anteriorly upon flexion. The disease is described as involving unilateral upper extremity with a distal-onset. We reported weakness and atrophy of the proximal part of an extremity in a 45-year-old man who is diagnosed with Hirayama disease. Proximal onset is a rare type of Hirayama Disease. Clinicians must be alert of proximal involvement and the diagnosis should be confirmed with electrophysiological and flexion MRI studies.


  1. Sakarya University Training and Research Hospital, Department of Neurology, Sakarya, Turkey
  2. Sakarya University Training and Research Hospital, Department of Radiology, Sakarya, Turkey



Further articles in this publication

Clinical Neuroscience

Can high uric acid levels be an independent risk factor for acute ischemic stroke due to large-artery atherosclerosis?

ACAR Türkan, ARAS Guzey Yesim, GÜL Sinem Sidika, ACAR Atılgan Bilgehan

Introduction - Uric acid is a molecule that is known to act as a natural antioxidant in acute oxidative stress conditions such as acute ischemic stroke (AIS). Although there are several studies on the prognostic value of serum uric acid (UA) level, especially the AIS, its importance in ischemic stroke is still controversial. Our aim in this study is to investigate whether the serum UA level is an indicative biomarker in the large-artery atherosclerosis in the AIS etiology. Material and method - Of the patients admitted to Sakarya University Training and Research Hospital Depart-ment of Neurology between January 2017 and November 2017, 91 hospitalized patients, who had AIS diagnosis and had their uric acid levels measured, were analyzed retrospectively. Patients with diabetes mellitus (DM), hypertension (HT), smoking habit, obesity, gout, hyperlipidemia (HL) and renal failure were excluded from the study. Patients were classified as anterior system and posterior system infarct. Then, patients were divided into two groups, one with internal carotid artery (ICA) > 50% stenosis and the other with ICA < 50% stenosis according to carotid-vertebral artery doppler USG examination performed for etiology. Serum UA, total bilirubin, direct bilirubin and indirect bilirubin levels of both groups were statistically compared. Results - In the comparison of serum UA values of ICA>50% stenosis and ICA<50% stenosis group of AIS patients, a statistically significant difference was found between the UA levels (p<0.000), but there was no difference between total bilirubin, direct bilirubin and indirect bilirubin values (p>0.05). Conclusion - High uric acid levels can be considered an independent, indicative risk factor for large-artery disease in AIS.

Clinical Neuroscience

[Paradigm shift in management of patients with vertigo and imbalance]

MIKE Andrea, TAMÁS T. László

[Dizziness is one of the most common causes of medical visits. Management of the dizzy patient may be challenging both for the general practitioner, in emergency departments, and special clinics, as behind a seemingly homogeneous clinical presentation several very different etiologies may occur. Research of the last two century enriched our knowledge about physiology and pathophysiology of the vestibular system. Much knowledge is now available about the labyrinth being able to sense head motions and gravity, processing of afferent vestibular stimuli, reflectory oculomotor and postural control, or recovery of the vestibular system. Based on scientific results new beside tests have been introduced including provocation maneuvers for detecting ectopic otoliths in different semicircular canals, head impulse test to examine function of the vestibulo-ocular reflex, and the HINTS+ battery for differentiation of peripheral or central origin of an acute vestibular syndrome. Technical innovations like videooculography and vestibular evoked myogenic potentials enable us to selectively and side-specifically examine the function of all six semicircular canals and two otolith organs. Pathomechanism of disorders with vertigo and dizziness became more clear resulting in the development or amendment of diagnostic criteria of several vestibular disorders including vestibular migraine, Menière’s disease, benign paroxysmal positional vertigo, persistent postural-perceptual dizziness, bilateral vestibulopathy. Broader knowledge about the pathomechanism promoted the development of new therapeutic methods like different repositioning maneuvers in benign paroxysmal positional vertigo, pharmaceutical therapies, vestibular rehabilitation, and psychotherapy. We aimed to summarize the novelties in the field of oto-neurology.]

Clinical Neuroscience

[Strategies of using the new antiepileptic drugs for epilepsy in adults]

NIKL János

[The new antiepileptic drugs have not changed the basic pharmacological treatment principles of epilepsy, but they have given greater choice in focal and in generalized epilepsies as well. The new drugs are not necessarily more effective than traditional drugs, but they have favourable pharmacokinetic characteristics, fewer interactions and better adverse effect profile in the acute and chronic phase of the treatment. They generally show a lower teratogenicity risk than the standard antiepileptics, although carbamazepine, one of the standard drugs can be used and zonisamide, a new one must be avoid in pregnancy. Due to characteristics mentioned above they are not only effective as add-on therapy, but in monotherapy as well. On the basis of the international and national recommendation lamotrigine and levetiracetam belong to the first line antiepileptics. The favourable tolerability of the new antiepileptics may improve the patient’s compliance and adherence to the given treatment. The low teratogenicity makes them especially suitable for the treatment of women of childbearing age. The new antiepileptic drugs can succesfully used for the treatment of special patients’ groups as for the post stroke, poszttraumatic epilepsies, for the epilepsies accompanied with brain tumours as well as for epilepsies in the elderly. The new drugs are advantageous for the treatment of such patients who have psychiatric symptoms or signs of cognitive decline and high risk of these symptoms respectively.]

Clinical Neuroscience

Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences


Purpose - To develop an evidence-based, standardized structured reporting (SR) method for brain MRI examinations in neonatal hypoxic-ischemic encephalopathy (HIE) suitable both for clinical and research use. Materials and methods - SR template development was based on comprehensive review of the pertinent literature with the basic sections and subdivisions of the template defined according to MRI sequences (both conventional and diffusion-weighted, MR-spectroscopy (MRS), and T2*-weighted imaging), and the items targeted on age-related imaging patterns of HIE. In order to evaluate the usability of the proposed SR template we compared data obtained from the brain MR image analysis of 87 term and 19 preterm neonates with the literature. The enrolled 106 infants were born between 2013 and 2015, went through therapeutic hypothermia according to the TOBY criteria due to moderate to severe asphyxia and had at least one brain MRI examination within the first two weeks of life. Ethical approval was obtained for this retrospective study. Descriptive statistical analysis was also performed on data exported from the structured reporting system as feasibility test. Results - The mean gestational age of the study population was 38.3±2.2 weeks; brain MRI was performed on 5.8±2.9 day of life, hence in 78% of our patients after the conclusion of therapeutic hypothermia. Our main imaging findings were concordant to the pertinent literature. Moreover, we identified a characteristic temporal evolution of diffusion changes. Interestingly 18% (n=19/106) of the clinically asphyxiated infants had isolated axial-extraaxial haemorrhage without any imaging sign of HIE. Conclusion - In this article our approach of reporting HIE cases with our novel SR template is described. The SR template was found suitable for reporting HIE cases, moreover it uncovered time and location dependent evolution of diffusion abnormalities (and pseudonormalization, as well), suggesting its usefulness in clinical research applications. The high number of isolated intracranial haemorrhages, and the changing diffusion pattern emphasizes the importance of early imaging in HIE.

Clinical Neuroscience

[Online supplementay figure to the article titled Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences]


[Online supplementay figure to the article titled Novel structured MRI reporting system in neonatal hypoxic-ischemic encephalopathy - issues of development and first use experiences]

All articles in the issue

Related contents

Clinical Neuroscience

[Magnetic resonance imaging in the course of alemtuzumab and teriflunomide therapy]

MIKE Andrea, KINCSES Zsigmond Tamás, VÉCSEI László

[Our work aimed to review the published results of magnetic resonance imaging (MRI) obtained in the course of alemtuzumab and teriflunomide therapy in multiplex sclerosis. In multiplex sclerosis MRI sensitively detects subclinical pathological processes, which do not manifest clinically in the early course of the disease, however have substantial significance from the viewpoint of the long-term disease prognosis. MRI has an increasingly important role in the early monitoring of the therapeutic efficacy. In the last 15 years several clinical trials have been conducted with alemtuzumab and teriflunomide in multiple sclerosis providing evidence about the favourable clinical effect of these drugs. MRI images were acquired in these trials as well, and the results published recently in the scientific literature. These MRI results denote the suppression of the disease activity and the neurodegenerative processes, which may imply a favourable effect on the long-term prognosis of the disease. ]

Clinical Neuroscience

[Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report]

ROSDY Beáta, KOLLÁR Katalin, MÓSER Judit, VÁRALLYAY György, KORDÁS Mariann

[Spontaneous intracranial hypotension is a rare complication of connective tissue disorders. One of them is Marfan syndrome. It predisposes the patient to meningeal diverticula. Possibly after minor unrecognised head trauma or secondary to Valsalva manoeuvre cerebrospinal fluid leak from meningeal diverticula can happen. It causes postural headache. We describe a 15 year old adolescent female newly diagnosed with Marfan syndrome, who presented with intractable postural headache. Our patient's brain MRI showed bilateral frontal subdural effusion, narrow ventricles, downward displacement of the brainstem, enlarged cervical venous plexi, dural ectasias and wide nerve root sleeves. Radionuclide cisternography demonstrated CSF leaks at multiple sites. She could not be treated conservatively, but was successfully treated by epidural saline injections. Control brain and cervical MRI confirmed her healing, too. At the two and half year follow up visit, she was completely well. So we recommend this easiest technic to use before epidural autolog blood patches.]

Clinical Neuroscience

[Neurological and psychiatrical prospects of apathy]


[During his long practice as head physician of a neurological and psychiatrical department with over 100 beds performed the examination and department of more than a hundred thousand patients. Based on the acquired experience and the data of the most recent literature he treats every aspect of the apathy syndrome. He emphasizes the multidisciplinary approach during both establishing the causes and the examination and treatment of patients. In order to clarify the diagnosis consultations with other disciplines must be used as well as the the knowledge provided by the now essential CT, MRI, PET, SPECT. The author discusses the international therapeutical possibilities and practice after the recently alredy possible exact diagnosis.]

Hungarian Radiology

[High resolution sonography for the examination of peripheral nerves]


[High-frequency sonography is an important method for the imaging of the peripheral nerves, even though it is rarely used. For the examination of superficially located nerves, currently available transducers with frequencies between 12-17 MHz offer a better axial resolution than even MRI. Sonography is superior to MRI especially for the examination of nerves of the upper extremity. Main indications for the sonography of the nerves are entrapment syndromes, traumatic injuries of the nerves, tumors, polyneuropathies and sonographically-guided interventions. The sensitivity of sonography and electrophysiology in the diagnosis of carpal tunnel syndrome and cubital tunnel syndrome are comparable. The combination of ultrasonography with electrophysiological studies increases the diagnostic yield in carpal and cubital tunnel syndromes. Sonography provides information for planning of peripheral nerve surgery and is helpful in evaluating postoperative complications. In selected cases, sonography can detect nerve lesions that require operative therapy earlier than electrophysiology. With technical enhancements, highfrequency ultrasonography is gaining increasing importance in the routine diagnostics of peripheral nerves lesions.]

Clinical Neuroscience

[Regulation of water transport in brain oedema]

DÓCZI Tamás, SCHWARCZ Attila, GALLYAS Ferenc, BOGNER Péter, PÁL József, SULYOK Endre, GÖMÖRI Éva, VAJDA Zsolt

[The study gives an overview on the regulation of cerebral water content and of brain volume. The molecular mechanisms of the development and resolution of various oedema forms are discussed in detail. The physiological and pathophysiological role of the recently discovered molecular water channel proteins aquaporin-1 (AQP1) and aquaporin-4 (AQP4) as well as the importance of central neuroendocrine regulation by vasopressin and atriopeptin are reviewed based on the relevant literature and personal studies. Quantitative water maps based on the combination of multicompartment- T2, diffusion weighted MRI and T1 studies have proven to be powerful tools for studying new drugs against brain oedema brought about by various neuropathological conditions and for testing their efficacy both in animal experimental and clinical conditions. Non-peptide vasopressin antagonists, atriopeptin agonists and drugs targeting AQP4 are potential new families of oedema-decreasing drugs.]