The fourth update of the rules of the procedure to determine the final, non-recoverable failure of the overall function of the cerebrum, the cerebellum and the brain stem, confirms the importance of somatosensory evoked potentials (SEP) and the early auditory evoked potentials (EAEP) for determination of irreversibility. This paper addresses the reliability and validity of the electrophysiological diagnosis, discusses the changes in the 4(th) version of the guidelines and shows up problems and sources of error in the practical application. The EAEP are valid for the irreversibility of brain death syndrome only in serial examinations or in the rare cases of sustaining wave I or wave I and II. Often an investigation is not reliable due to possible existing sound conduction disturbances or failure of all potentials before entering the clinical brain death syndrome. We therefore use the EAEP only in exceptional cases. The median nerve SEP are reliably derivable, technically simple and with few errors. There is no serial investigation required and the time required for deriving is low.

Symptoms related to lesions of the peripheral nervous system (PNS) may be the initial clinical manifestation of a previously unknown tumor, arising from either the nerve tissue (e. g., schwannoma) or – more common – from non-neurogenic structures, such as lung cancer in pancoast tumors, metastatic lymph node infiltrations or lymphomas.

Cubital tunnel syndrome (CuTS) is the second most common peripheral nerve compression syndrome. In German-speaking countries, cubital tunnel syndrome is often referred to as sulcus ulnaris syndrome (retrocondylar groove syndrome). This term is anatomically incorrect, since the site of compression comprises not only the retrocondylar groove but the cubital tunnel, which consists of 3 parts: the retrocondylar groove, partially covered by the cubital tunnel retinaculum (lig. arcuatum or Osborne ligament), the humeroulnar arcade, and the deep flexor/pronator aponeurosis. According to Sunderland , cubital tunnel syndrome can be differentiated into a primary form (including anterior subluxation of the ulnar nerve and compression secondary to the presence of an anconeus epitrochlearis muscle) and a secondary form caused by deformation or other processes of the elbow joint. The clinical diagnosis is usually confirmed by nerve conduction studies. Recently, the use of ultrasound and MRI have become useful diagnostic tools by showing morphological changes in the nerve within the cubital tunnel. A differential diagnosis is essential in atypical cases, and should include such conditions as C8 radiculopathy, Pancoast tumor, and pressure palsy. Conservative treatment (avoiding exposure to external noxes and applying of night splints) may be considered in the early stages of cubital tunnel syndrome. When nonoperative treatment fails, or in patients who present with more advanced clinical findings, such as motor weakness, muscle atrophy, or fixed sensory changes, surgical treatment should be recommended. According to actual randomized controlled studies, the treatment of choice in primary cubital tunnel syndrome is simple in situ decompression, which has to be extended at least 5-6 cm distal to the medial epicondyle and can be performed by an open or endoscopic technique, both under local anesthesia. Simple decompression is also the therapy of choice in uncomplicated ulnar luxation and in most post-traumatic cases and other secondary forms. When the luxation is painful, or when the ulnar nerve actually "snaps" back and forth over the medial epicondyle of the humerus, subcutaneous anterior transposition may be performed. In cases of severe bone or tissue changes of the elbow (especially with cubitus valgus), the anterior transposition of the ulnar nerve may again be indicated. In cases of scarring, submuscular transposition may be preferred as it provides a healthy vascular bed for the nerve as well as soft tissue protection. Risks resulting from transposition include compromise in blood flow to the nerve as well as kinking of the nerve caused by insufficient proximal or distal mobilization. In these cases, revision surgery is necessary. Epicondylectomy is not common, at least in Germany. Recurrence of compression on the ulnar nerve at the elbow may occur. This review is based on the German Guideline "Diagnose und Therapie des Kubitaltunnelsyndroms" ( www.leitlinien.net ).

The cubital tunnel syndrome is one of the most widespread compression syndromes of a peripheral nerve. In German-speaking countries it is known as the sulcus ulnaris syndrome (retrocondylar groove syndrome), which is anatomically incorrect. The cubital tunnel consists of the retrocondylar groove, the cubital tunnel retinaculum (Lig. arcuatum or Osborne band), the humeroulnar arcade and the deep flexor/pronator aponeurosis. According to Sunderland it can be divided into a primary form (including the ulnar luxation and the epitrocheoanconaeus muscle) and a secondary form caused by deformation or other processes of the elbow joint. The diagnosis has to be confirmed by a thorough clinical examination and nerve conduction studies. Neurosonography and MRI are becoming more and more important with improving resolution and enable the direct identification of morphological changes. Differential diagnosis is essential in atypical cases, especially C8 syndrome and pressure palsy. Double crush (double compression syndrome) may occur. Operative treatment is more effective than conservative treatment, which consists primarily of the prevention of exposure to external noxes. According to actual randomised controlled studies the therapy of choice of the primary form in most cases is the simple in situ decompression of the ulnar nerve in the cubital tunnel. This has to be extended at least up to 5-6 cm distally of the medial epicondyle and can be performed in the open or endoscopic technique, both under local anesthesia. Simple decompression is also the therapy of choice in uncomplicated ulnar luxation and in most post-traumatic cases and other secondary forms. In cases of severe bony or tissue changes of the elbow (especially cubitus valgus) the volar transposition of the ulnar nerve may be indicated. This can be performed in a subcutaneous or submuscular technique. Risks of transposition are impairment of perfusion and, above all, kinking caused by insufficient proximal or distal mobilisation of the nerve has to be avoided. In these cases revision surgery is necessary. The epicondylectomy is not common in our country. Recurrences may occur.

The cubital tunnel syndrome is one of the most widespread compression syndromes of a peripheral nerve. In German-speaking countries it is known as the sulcus ulnaris syndrome (retrocondylar groove syndrome), which is anatomically incorrect. The cubital tunnel consists of the retrocondylar groove, the cubital tunnel retinaculum (Lig. arcuatum or Osborne band), the humeroulnar arcade and the deep flexor/pronator aponeurosis. According to Sunderland [159] it can be divided into a primary form (including the ulnar luxation and the epitrocheoanconaeus muscle) and a secondary form caused by deformation or other processes of the elbow joint. The diagnosis has to be confirmed by a thorough clinical examination and nerve conduction studies. Neurosonography and MRI are becoming more and more important with improving resolution and enable the direct identification of morphological changes. Differential diagnosis is essential in atypical cases, especially C8 syndrome and pressure palsy. Double crush (double compression syndrome) may occur. Operative treatment is more effective than conservative treatment, which consists primarily of the prevention of exposure to external noxes. According to actual randomised controlled studies the therapy of choice of the primary form in most cases is the simple in situ decompression of the ulnar nerve in the cubital tunnel. This has to be extended at least up to 5-6 cm distally of the medial epicondyle and can be performed in the open or endoscopic technique, both under local anesthesia. Simple decompression is also the therapy of choice in uncomplicated ulnar luxation and in most post-traumatic cases and other secondary forms. In cases of severe bony or tissue changes of the elbow (especially cubitus valgus) the volar transposition of the ulnar nerve may be indicated. This can be performed in a subcutaneous or submuscular technique. Risks of transposition are impairment of perfusion and, above all, kinking caused by insufficient proximal or distal mobilisation of the nerve has to be avoided. in these cases revision surgery is necessary. The epicondylectomy is not common in our country. Recurrences may occur.

The cubital tunnel syndrome is one of the most widespread compression syndromes of a peripheral nerve. In German-speaking countries it is known as the sulcus ulnaris syndrome (retrocondylar groove syndrome), which is anatomically incorrect. The cubital tunnel consists of the retrocondylar groove, the cubital tunnel retinaculum (Lig. arcuatum or Osborne band), the humeroulnar arcade and the deep flexor/pronator aponeurosis. According to Sunderland it can be divided into a primary form (including the ulnar luxation and the epitrocheoanconaeus muscle) and a secondary form caused by deformation or other processes of the elbow joint. The diagnosis has to be confirmed by a thorough clinical examination and nerve conduction studies. Neurosonography and MRI are becoming more and more important with improving resolution and enable the direct identification of morphological changes. Differential diagnosis is essential in atypical cases, especially C8 syndrome and pressure palsy. Double crush (double compression syndrome) may occur. Operative treatment is more effective than conservative treatment, which consists primarily of the prevention of exposure to external noxes. According to actual randomised controlled studies the therapy of choice of the primary form in most cases is the simple in situ decompression of the ulnar nerve in the cubital tunnel. This has to be extended at least up to 5-6 cm distally of the medial epicondyle and can be performed in the open or endoscopic technique, both under local anesthesia. Simple decompression is also the therapy of choice in uncomplicated ulnar luxation and in most post-traumatic cases and other secondary forms. In cases of severe bony or tissue changes of the elbow (especially cubitus valgus) the volar transposition of the ulnar nerve may be indicated. This can be performed in a subcutaneous or submuscular technique. Risks of transposition are impairment of perfusion and, above all, kinking caused by insufficient proximal or distal mobilisation of the nerve has to be avoided. In these cases revision surgery is necessary. The epicondylectomy is not common in our country. Recurrences may occur.

Background: Posterior tarsal tunnel syndrome is an uncommon clinical entity which is sometimes misdiagnosed in patients with pain of the retromalleolar region and the plantar aspect of the foot. Surgical intervention is recommended for correctly diagnosed posterior tarsal tunnel syndrome.Methods: Selective literature review.Results: Surgical treatment is indicated in the presence of dysesthesias refractory to conservative treatment or of neurological deficits. If a neural tumor or tarsal tunnel ganglion is suspected, diagnostic imaging (MRI, neurosonography) should precede surgery. Division of the flexor retinaculum (ligamentum laciniatum) in the tarsal tunnel must always include distal decompression of the end branches of the tibial nerve posterior to the fascia of the abductor hallucis muscle. Only extensive exposure of the nerve guarantees adequate release.Conclusion: Accurate diagnosis requires the evaluation of relevant clinical, neurological, and neurophysiological findings along with the careful consideration of other possible diagnoses. High success rates of 44% to 91% are reported after operative treatment. The results are better in idiopathic than in posttraumatic cases. If surgery fails, re-operation is indicated only in patients with inadequate release.

Evidence-based supradisciplinary guideline that deals with the epidemiology, pathogenesis, symptoms, clinical and electrophysiological diagnosis, supplementary imaging investigations, differential diagnosis, conservative and surgical treatments, prognosis and course along with complications and revision surgery. The recommendations on investigation and treatment are based on a comprehensive literature search with critical evaluation and two consensus methods (expert group and Delphi technique) within the participating specialist societies. Besides this long version, a short version and a patient version can be viewed through the AWMF platform. The development of the guideline and the methodological foundations are documented in a method report. MAIN STATEMENTS: Apart from an accurate history and clinical neurological examination (including clinical tests), electrophysiological investigations (distal motor latency and sensory neurography) are particularly important. Radiography, MRI, high-resolution ultrasonography can be regarded as optional supplementary investigations. Among conservative treatment methods, treatment with a nocturnal splint and local infiltration of a corticosteroid preparation are effective. Oral steroids, splinting and ultrasound showed only short-term benefit. Surgical treatment is clearly superior to all other methods. Open and endoscopic procedures (when the endoscopic surgeon has sufficient experience) are equivalent. A routine epineurotomy and interfascicular neurolysis cannot be recommended. Early functional treatment postoperatively is important.

Background: Andermann syndrome (OMIM 218000) is an autosomal recessive motor-sensory neuropathy associated with developmental and neurodegenerative defects. The cerebral MRI reveals a variable degree of agenesis of the corpus callosum. Recently, truncating mutations of the KCC3 gene (also known as SLC12A6) have been associated with Andermann syndrome. Methods: The authors assessed clinically and genetically three isolated cases from Germany and Turkey with symptoms consistent with Andermann syndrome. Results: The authors detected four novel mutations within the KCC3 gene in their patients: two different truncating mutations in the first patient, a homozygous truncating mutation in the second, and a homozygous missense mutation in the third patient. In contrast to the classic phenotype of the Andermann syndrome linked to truncating KCC3 mutations the phenotype and the course of the disease linked to the missense mutation appeared to be different (i. e., showing additional features like diffuse and widespread white matter abnormalities). Conclusions: Not only truncating but also missense mutations of the KCC3 gene are associated with Andermann syndrome. Different types of KCC3 mutations may determine different clinical phenotypes.

Introduction: Septic encephalopathy (SE) and critical-illness polyneuropathy (CIP) are neurological manifestations of the sepsis syndrome and worsen the prognosis. Somatosensory evoked potential (SEP) testing is a widely used method to detect dysfunction of the peripheral and central nervous system in ICU patients. In this study the type, extent and reversibility of SEP abnormalities were studied in respect to its diagnostic and prognostic significance in patients with neurological complications of sepsis. Patients and method: SEP testing was done in 100 patients with sepsis and neurological complications (80% weaning failure) including recordings from the plexus brachialis, the cervical medulla and the somatosensory cortex. In 36 patients the time course could be studied. Results: The PNS lesions were caused by an axonal PNP. Isolated abnormalities of the PNS were found in 13 (17)%, of the CNS in 31(17) % and a combination of peripheral and central abnormalities in 50 (63) % of patients with single and (repeated) examinations. In more than 75 % the abnormalities were symmetric. The most striking finding was a reversible increase of the N20/P25 (> 10 mu V in 34 %) and preserved cortical SEP in all other patients and even those with absent peripheral potentials. Conclusion: SEP demonstrate mainly symmetrical abnormalities in the PNS and CNS. This is in line with the concept considering SE and CIP as part of the septic multiorgan failure. Although SEP do not contribute to the prognosis the combination of a symmetric axonal PNP and significantly enhanced cortical responses seems to be a typical diagnostic finding in ICU patients with sepsis.

Sporadic inclusion body myositis is a chronic progressive inflammatory myopathy leading to paresis and atrophy of distal and proximal muscles. We present the case of an 80-year old woman with slowly progressive weakness and atrophy of her left leg and right hand. At the time of investigation the distal muscles involved were finger flexors and extensors of the feet, the proximal muscles were extensors of the knees and flexors of the elbows. Needle electromyographic examination of the right biceps brachii showed a normal mean duration and amplitude of motor unit potentials (MUP), but also a few short and low MUP. There was an increased rate of polyphasic potentials of 50%. The left vastus medialis showed the typical pattern of a rnyopathic process with a decrease in mean amplitude and duration of MUP. It was very unusual that all muscles investigated showed myotonic discharges. Muscle biopsy agreed with the diagnosis of inclusion body myositis. The presented case demonstrates that sporadic inclusion body rnyositis is an important differential diagnosis in an elderly patient with asymmetric paresis and muscular atrophy.