[Diagnosis and therapy of carpal tunnel syndrome--guideline of the German Societies of Handsurgery, Neurosurgery, Neurology, Orthopaedics, Clinical Neurophysiology and Functional Imaging, Plastic, Reconstructive and Aesthetic Surgery, and Surgery for Traumatology]. / Diagnostik und Therapie des Karpaltunnelsyndroms--Leitlinie der Deutschen Gesellschaften für Handchirurgie, Neurochirurgie, Neurologie, Orthopädie unter Mitbeteiligung der Gesellschaften für Unfallchirurgie, Klinische Neurophysiologie und Funktionelle Bildgebung sowie Plastiche, Rekonstruktive und Asthetische Chirurgie.

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.

Retrograde changes of nerve fibers with the carpal tunnel syndrome. An electroneurographic investigation.

In the CTS the motor nerve conduction velocity proximal to the wrist is reduced in proportion to the degree of severity of the nerve lesion. Furthermore the evoked nerve action potential is significantly reduced when recordings are made from the median nerve at the elbow and when the compound nerve is stimulated proximal to the lesion at the wrist. The extent of the retrograde changes correlates with the degree of severity and duration of nerve compression. Measurement of the evoked nerve action potential in the proximal nerve segment enables us to estimate the extent of the retrograde nerve fiber degeneration and therefore might be important for prognosis.

Cubital tunnel syndrome - a review and management guidelines.

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 ).

[Diagnosis and therapy of cubital tunnel syndrome--state of the art]. / Aktueller Stand der Diagnostik und Therapie des Kubitaltunnelsyndroms.

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.

Novel truncating and missense mutations of the KCC3 gene associated with Andermann syndrome.

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.

Somatosensory evoked potentials following trigeminal nerve stimulation in trigeminal neuralgia.

The examination of somatosensory evoked potentials following trigeminal nerve stimulation in 20 subjects with idiopathic trigeminal neuralgia revealed a pathological increase in latency of the first positive component on the involved side in 7 (41%) of the 17 patients without antecedent surgical treatment. The mean difference in latency between the two sides was 1.3 msec (2p greater than or equal to 0.005). These results are indicative of microtraumatization of the trigeminal nerve in the posterior fossa by blood vessels, for example, leading to local demyelinization. The method seems to be of diagnostic value in selecting patients for more decompression by posterior fossa operations.

[Alopecia in meralgia paraesthetica]. / Alopezie bei der Meralgia paraesthetica.

Two patients are described who had a lesion in the lateral cutaneous nerves of the thigh, both of which were verified by anamnestic, clinical and electrophysiological data. Both showed alopecia in the region supplied by the nerve. A biopsy performed on one patient showed atrophy of the epidermis and hair follicles. It is pointed out that this kind of trophic disturbance, which is very seldom described in literature on meralgia paraesthetica, may possibly be found more often in long-standing cases of this disease.

[Electromyographic signs of neurogenic involvement in hypokalemic paralysis as a result of diuretic abuse]. / Elektromyographische Zeichen einer neurogenen Beteiligung bei hypokaliämischer Lähmung infolge Diuretika-Abusus.

A female is reported, which showed motor and sensory disturbances following severe hypokalemia induced by abuse of diuretics. Elevated CSF-protein and abnormal neurographic findings with restitution after substitution with potassium led to the diagnosis of a neurogenic involvement like a Guillain-Barré syndrome. In the literature similar cases are described. So it seems, that beside of myopathic changes neurogenic disturbances can appear as a neurological symptom of hypokalemia.

[Polyneuropathy following treatment of chronic hepatitis B with vidarabine]. / Polyneuropathie bei Behandlung der chronischen Hepatitis B mit Vidarabin.

Two patients with chronic hepatitis B received adenine arabinoside, at a dosage of 1000 mg/d for 8 weeks and 700 mg/d for 12 weeks, respectively. At the end of the treatment period both patients had developed severe sensory irritability in the distal parts of the legs: perception of all sensory modalities was impaired. The disorder persisted over the following three years, although in a milder form. Shortly after the onset of the symptoms sensory nerve conduction velocity in both lower legs was not definitely impaired. But six months later it was clearly reduced to below 40 m/sec in the sural nerve, a finding which has persisted a year later. Three years after the first examination both patients are slightly improved, but conduction velocity is not yet normal. The objectively demonstrated long course and the considerable subjective complaints associated with the polyneuropathy argue for a very therapeutic use of adenine arabinoside.

[EMG and evoked potentials in adrenomyeloneuropathy]. / EMG und evozierte Potentiale bei der Adrenomyeloneuropathie.

In a 31 year old patient with adrenocortical gland insufficiency and minor clinical signs of involvement of peripheral and central nervous system an adrenoleucodystrophy was diagnosed, based upon the characteristic elevation of hexacosanoic acid in plasma. The motor and sensory nerve conduction velocities in upper and lower extremities were markedly reduced; in accordance, the somatosensory evoked potentials showed distinct reduction in the peripheral part. In contrast, the interpeak latencies N 20 - NSEP 3 (median nerve) and N 22 - P 40 (tibial nerve) were in the upper normal range, so that a damage of the central sensory system could not be evaluated. The visual and brainstem acoustic evoked potentials were a little prolonged. The problems in differential diagnosis of this form of adrenoleucodystrophy, which especially involves the peripheral nervous system, are discussed.

[Hashimoto encephalopathy: steroid-sensitive encephalopathy in Hashimoto thyroiditis]. / Hashimoto-Enzephalopathie. Steroid-sensitive Enzephalopathie bei Hashimoto-Thyreoiditis.

Hashimoto's encephalopathy is a steroid-responsive, relapsing or progressive encephalopathy associated with Hashimoto's thyroiditis. Characteristic clinical features are confusion, seizures, alteration in conscious level, stroke-like episodes, myoclonus, and tremulousness. High CSF protein levels without pleocytosis and a diffusely abnormal EEG are typical findings. Brain CT and MRI and cerebral angiogram are usually normal. We present two case reports of Hashimoto's encephalopathy in 55- and 77-year-old patients who both responded well to steroid therapy, and review the literature.