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Background: The treatment of autoimmune disorders of the nervous system is based on interventions for the underlying immune phenomena., Objective: To summarize concepts of cell depletion and myeloablation studied in the context of neuroimmunological disorders., Method: Evaluation of the available literature on multiple sclerosis as the most widely studied neuroimmunological entity., Results: Three concepts have been introduced: classical immunosuppressants, such as azathioprine, mitoxantrone and cyclophosphamide exert general lymphopenic effects and thereby moderately decrease disease activity. Myeloablative regimens combined with autologous hematopoietic stem cell transplantation have a profound and in most cases long-lasting impact on autoimmunity at the cost of potentially life-threatening side effects. Alemtuzumab (anti-CD52), rituximab and ocrelizumab (both anti-CD20) are depleting antibodies directed against certain lymphocyte subsets and substantially ameliorate disease activity in relapsing-remitting multiple sclerosis. Ocrelizumab also shows efficacy in the primary progressive form of multiple sclerosis., Conclusions: Most of the presented cell-depleting and myeloablative therapies are highly effective treatment options but are also accompanied by significant risks. In the context of the increasing number of alternative immunomodulatory options the indications for use should be cautiously considered.

A 43-year-old patient presented to our department because her left eyelid had exhibited drooping to varying extents for 2-3 months. Furthermore, in extreme positions or after rapid eye movement, she perceived diplopic images and was sensitive to light. The diagnosis of sphenoid meningioma was reached. Subsequently two rounds of radiopeptide therapy with (90)Y-DOTATOC (somatostatin analog) were administered within 3 months. This treatment approach led to a reduction of tumor volume in our patient as well as clinical improvement followed by stabilization.

Purpose: Magnetic resonance imaging (MRI) has become a valuable tool for diagnosing and monitoring multiple sclerosis (MS). The high sensitivity for the detection of hyperintense lesions in T 2-weighted scans contributes substantially to diagnosis. The initial lesion number or lesion volume stands for an increased probability of further accumulation of lesion burden, an earlier conversion to clinically definite MS and progression of disability in the next 5 - 15 years. This diagnostic and prognostic information gained from MRI early in the disease course lead in 2001 to a revision of the diagnostic criteria., Materials and Methods: For the first time MRI criteria were defined in addition to the clinical and paraclinical criteria using the clinical terms for dissemination with respect to space and time. In particular, the defined MRI criteria are based on lesion number and location, the appearance of new lesions and lesion enhancement using contrast agent., Results: Reliable detection and description of older and new lesions in the disease course by MRI represents subclinical disease activity which can substitute the clinical confirmation of a relapse leading to an earlier diagnosis. This places importance on the assessment of the subclinical disease activity in sequential MR scans requiring a standardized and reproducible approach to minimize variability despite different MR scanners., Conclusion: This review provides an updated proposal for the approach and management of cranial and spinal MR scans in patients with MS. We describe the influence of variables which cannot be standardized (scanner, field strength, manufacturer and software) and outline potential pitfalls of clinical MR imaging in MS resulting from a non-standardized approach. This updated proposal for slice positioning, sequences and documentation is a result of a consensus process targeting systematic and standardized use in clinical MR evaluations of MS.

History and Clinical Findings: A 34-year-old previously healthy woman presented with a five-day history of subacute onset of a numb chin. Examination on admission revealed isolated hypesthesia on the left side of the chin and lower lip., Investigations: Brain magnetic resonance imaging (MRI) demonstrated a lesion involving the pontine trigeminal fibers and multiple periventricular T2-hyperintense white matter lesions suggestive of inflammatory /demyelinating disease. Cerebrospinal fluid analysis revealed oligoclonal IgG bands (only in cerebrospinal fluid) and an increased IgG index. A follow-up MRI after four months demonstrated new supratentorial brain lesions, confirming a syndrome, highly suggestive of multiple sclerosis as the likely underlying diagnosis. TREATMENT AND FOLLOW-UP: The facial sensory disturbance resolved spontaneously. Prophylactic treatment with interferon-beta was started., Conclusion: The numb chin syndrome may be the initial presentation of a clinically isolated syndrome suggestive of multiple sclerosis. Prophylactic immunomodulatory treatment may be started after the suspicion of inflammatory/demyelinating activity is confirmed.

Radiotherapy of breast cancer, cervical cancer, testicular tumours and lymphoma is one of the most effective therapy options. Damage to the nervous system, in particular the brachial and lumbar plexus, is rare and typically leads to development of progressive sensory disturbances and motor weakness after years-long latency. We present two cases exemplifying the diagnostic problems in differentiating between radiation-induced injury and recurrence of the primary tumour. A clinical course with sensorimotor symptoms and signs progressing over months, electomyographic recording of myokymic discharges, and absence of a space-occupying mass suggest late-onset radiation-induced plexopathy. The literature on pathogenesis and incidence of radiation-induced plexopathy is reviewed.

About one third of patients with HIV infection show neurological complications with considerable morbidity and high mortality. This is an actualized review of the most important neurological manifestations resulting from primary HIV infection, from secondary opportunistic infections, or as complications of antiretroviral therapy. The primary neurological manifestations, including HIV-associated dementia complex, myelopathies, peripheral neuropathies and myopathies, the more common opportunistic infections, primary central nervous system lymphoma and cerebrovascular diseases, are discussed in the light of new evidence in diagnosis, therapy and prognosis. Cognitive and psychiatric symptoms, visual changes, headache, seizures, dizziness, involuntary movements, gait disturbances, cranial neuropathies and focal deficits are the common neurological symptoms in HIV infection which are described under the aspect of differential diagnosis. It is important to bear in mind that nearly all information available to date on this subject concerns HIV patients in the period before combination therapies (including protease inhibitors). The introduction of highly active antiretroviral therapy (HAART) with protease inhibitors in 1995, and non-nucleoside reverse transcriptase inhibitors, have opened up new therapeutic modalities with a new emphasis on earlier detection and treatment of neurological complications. The prognosis of different HIV-associated neurological diseases has considerably improved, as recently shown in the case, for example, of progressive multifocal leucoencephalopathy.

Magnetic resonance imaging (MRI) is highly sensitive to pathological tissue changes in multiple sclerosis (MS) patients. It demonstrates the frequently subclinical disease activity and follow-up examinations regularly show the accumulation of new lesions and the development of atrophy. The increasing importance of follow-up examinations in MS patients makes it necessary to provide comparable MRI data even over long observation periods. This review article focusses on critical variables in this regard and technical issues; practical guidelines for MRI protocols in MS patients are presented. The influence of field strength, MR systems from different manufacturers, and new software releases is described. Guidelines concerning the graphic planning of the examination, sequence protocols, documentation and reporting of cranial MR studies in MS patients are presented.

A 65 year old woman, referred for differential diagnosis of dementia, presented with cognitive and mnestic deficits. Typical signs and symptoms of hypothyroidism led to the diagnosis of severe autoimmune thyroiditis. After six months of treatment with thyroid hormone, dementia, myopathy and ataxia had disappeared. The neurologic and neuropsychologic manifestations of hypothyroidism are discussed. In dementia the diagnosis of secondary and reversible forms is of great importance with regard to prognosis and treatment. Hypothyroidism is known to be a common and treatable cause of pseudodementia. Early diagnosis and adequate treatment are important to prevent further permanent deterioration of cerebral function.

The diagnosis of multiple sclerosis (MS) relies on the occurrence of characteristic symptoms, on the patient's history and on the correct interpretation of nowadays very sensitive but not very specific auxillary examinations. This paper reviews the diagnostic criteria and typical signs and symptoms of the disease. The significance of cerebrospinal fluid (CSF) analysis, magnetic resonance imaging (MRI) and evoked potentials is discussed, with emphasis on their predictive value concerning the development of MS after a first episode with symptoms suggestive of MS. A wide range of other diseases mimicking MS, like infectious, autoimmune, granulomatous, metabolic and hereditary diseases is reviewed. A checklist for important points in patient history and further investigations is proposed.

Recombinant interferon beta-1b has been registered with the Swiss health authorities since August 1995. Due to a special arrangement with health insurances it has been possible to prescribe this medication since spring 1995. We report on our experience with the first 30 consecutively treated multiple sclerosis patients. Indication, adverse event profile and clinical response to treatment are described. The most common side effects were local injection site reactions (63%), influenza-like symptoms (50%) and fatigue (33%). As compared to the prestudy period we observed a 49% reduction in the exacerbation rate. Compliance was excellent, possibly due to strict selection and extensive information about possible effects and side effects.

The diagnosis of multiple sclerosis remains a clinical one. It is based on the documentation of lesions at two or more sites of the CNS and of two or more relapses in relapsing-remitting disease, or persistent deterioration for 6 months in primarily chronic-progressive disease. Because no symptom and no result of a paraclinical investigation is specific enough, a careful differential diagnostic work up by a physician competent in clinical neurology is essential. CSF examination, blood serology, MRI and in some cases evoked potentials may help in this work-up. The results of recently completed controlled trials suggest that, besides high dose steroid treatment of acute relapses, immunomodulatory treatments may help to improve the prognosis in relapsing-remitting phase of the disease. Treatments that will enter clinical practice in the next year or two are recombinant interferon beta-1b and -1a and perhaps copolymer 1. Non-selective immunosuppressants (azathioprine, in more severe cases other substances with more intensive cytostatic activity) also have a place in selected cases. The efficacy of such treatment in secondary chronic-progressive disease as well as the indication for very early treatment are now under investigation. Some possible future developments in diagnosis and therapy of MS are discussed.

Hereditary motor sensory neuropathies are a heterogeneous group of inherited diseases of the peripheral nerves. In this review the clinical and genetic differences between the sub-groups of this disease will be discussed. Since the discovery of a 1.5 mb duplication on chromosome 17 p11.2-12 in most patients with a hereditary motor sensory neuropathy and a variety of different mutations on chromosomes 1 and X in other patients with a similar disease profile, Dycks' clinical classification needs to be re-evaluated. In this review Dycks' taxonomy of heridihary neuropathies will be compared to a new genetic classification and a relevant diagnostic procedure proposed when a hereditary neuropathy is suspected.

We report on a 35-year old patient with multiple sclerosis (MS) with clinically and electrophysiologically typical facial myokymia, appearing during an acute bout of the disease. Magnetic resonance imaging (MRI) disclosed two new pontine lesions. During a follow-up period of eight months facial myokymia subsided, but five months later central fever with good therapeutic response to carbamazepine developed. In this report we discuss the potential mechanisms of myokymia and central temperature dysregulation in MS.