WITHDRAWN: MS related employment and disease modifying treatment in the German working population: 1994-2009.

Ahead of Print article withdrawn by publisher.

Postsynaptic nigrostriatal dopamine receptors and their role in movement regulation.

The article presents the hypothesis that nigrostriatal dopamine may regulate movement by modulation of tone and contraction in skeletal muscles through a concentration-dependent influence on the postsynaptic D1 and D2 receptors on the follow manner: nigrostriatal axons innervate both receptor types within the striatal locus somatotopically responsible for motor control in agonist/antagonist muscle pair around a given joint. D1 receptors interact with lower and D2 receptors with higher dopamine concentrations. Synaptic dopamine concentration increases immediately before movement starts.

Gasperini syndrome as clinical manifestation of pontine demyelination.

The Gasperini syndrome is a very rare brainstem disease characterized by the typical combination of ipsilateral lesions of the cranial nerves V-VII and dissociated contralateral hemihypesthesia, whereas both contralateral and ipsilateral hypacusis was described. Since the first description in 1912, only a few cases of this crossed brainstem syndrome were published so far. Pontine infarction and bleedings were the reported causes of the syndrome.

Puusepp's sign--clinical significance of a forgotten pyramidal sign.

The pyramidal signs in the lower extremity can be divided into three groups: (1) Babinski's group characterised by dorsoflexion of the great toe, (2) pyramidal signs marked by plantar flexion of the toes (e.g. Rossolimo's sign), and (3) synkinetic movements such as Strümpell's phenomenon.

Bruns syndrome caused by intraventricular tumor.

The Bruns syndrome is an unusual phenomenon, characterized by attacks of sudden and severe headache, vomiting and vertigo, triggered by abrupt movement of the head. The presumptive cause of the Bruns syndrome is a mobile deformable intraventricular lesion leading to an episodic obstructive hydrocephalus resulted from an intermittent or positional CSF obstruction with elevation of intracranial pressure due to a ball-valve mechanism. Although the old neurological literature recognized tumors as well as neurocysticercosis as causes of the Bruns syndrome, during the last 60 years only intraventricular neurocysticercosis was reported to cause this symptom-complex.

[Acute persistent bilateral loss of vision].

Bilateral severe vision loss is a dramatic illness that requires extended diagnostics and immediate therapy. We report on a 37-year-old man who was admitted with lower back pain, headache, and fever. Within 3 days he developed meningism, pleocytosis in the cerebral spinal fluid, and bilateral vision loss, with the vision loss occurring within a few hours.

Bruns syndrome caused by intraventricular tumor.

The Bruns syndrome is an unusual phenomenon, characterized by attacks of sudden and severe headache, vomiting and vertigo, triggered by abrupt movement of the head. The presumptive cause of the Bruns syndrome is a mobile deformable intraventricular lesion leading to an episodic obstructive hydrocephalus resulted from an intermittent or positional CSF obstruction with elevation of intracranial pressure due to a ball-valve mechanism. Although the old neurological literature recognized tumors as well as neurocysticercosis as causes of the Bruns syndrome, during the last 60 years only intraventricular neurocysticercosis was reported to cause this symptom-complex.

Between Wallenberg syndrome and hemimedullary lesion: Cestan-Chenais and Babinski-Nageotte syndromes in medullary infarctions.

In comparison with the lateral (Wallenberg), medial (Dejerine) and hemimedullary (Reinhold) medulla oblongata syndromes, the Babinski-Nageotte and Cestan-Chenais syndromes are much less familiar cerebrovascular disorders. While the Babinski-Nageotte syndrome is usually confused with the hemimedullary syndrome, reports of the extremely rare Cestan-Chenais syndrome are missing from the modern neurological literature. The pathological and magnetic resonance imaging (MRI) correlations of the Cestan-Chenais syndrome have not been shown so far.

Isolated bilateral blindness as the sole manifestation of transient ischaemic attacks.

Purpose: To explain the rare phenomenon of acute transient bilateral blindness without additional ophthalmological or neurological symptoms and signs.

Methods: Six patients with isolated bilateral visual loss lasting 1-15 mins and occurring simultaneously in both eyes were evaluated. Clinical observation, neuroimaging (CT, MRI, MR-angiography), extra- and transcranial Doppler and vascular risk factors assessment were performed.

Call-Fleming postpartum angiopathy in the puerperium: a reversible cerebral vasoconstriction syndrome.

Background: The postpartum angiopathy (Call-Fleming syndrome) is a rare, reversible cerebral vasoconstriction syndrome. Unlike in eclampsia, neither proteinuria nor hypertension have been observed in the Call-Fleming syndrome.

Case: A 17-year-old woman developed headache, seizures, confusion, cortical blindness, and denial of visual loss (optic anosognosia, Anton syndrome) on the first postpartum day.

Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies.

Previous findings suggested specific mitochondrial dysfunction in skeletal muscle of patients with amyotrophic lateral sclerosis (ALS). To answer the question of whether the dysfunction is specific, we investigated the histochemical distribution of mitochondrial marker activities, the ratio of mitochondrial (mt) versus nuclear (n) DNA, and the activities of citrate synthase (CS) and respiratory chain enzymes in muscle biopsies of 24 patients with sporadic ALS. The data were compared with those in 23 patients with other neurogenic atrophies (NAs), and 21 healthy controls.

Dilated cardiomyopathy may be an early sign of the C826A Fukutin-related protein mutation.

Limb-girdle muscular dystrophy LGMD2I is caused by mutations in the fukutin-related protein (FKRP) gene. Clinically, LGMD2I exhibits a great phenotypic variability ranging from severe, rapidly progressive weakness and wasting of the limb-girdle muscles to mild disorders. Here, we present three siblings without clinical signs of muscle dystrophy, but with dilated cardiomyopathy.

[Classical crossed pontine syndromes].

Definitions of classical crossed brainstem syndromes in the modern neurological literature are often inaccurate and inconsistent. As a result, different clinical syndromes are designated with the same eponym, other crossed syndromes are nearly completely forgotten. In this study, the original historical publications on the classical alternating pontine syndromes of Foville, Millard-Gubler, Raymond, Raymond-Cestan, Brissaud-Sicard, Gasperini, Grenet and Gelle were reviewed and critically analysed.

Serial magnet resonance angiography in patients with vasculitis and vasculitis-like angiopathy of the central nervous system.

The purpose of the study was to examine the value of the non-invasive magnet resonance angiography (MRA) in the follow-up of cerebral vasculitis (CV) and vasculitis-like angiopathy. We performed follow-up MRA (TOF 3D), MRI and transcranial doppler ultrasound (TCD) in the patients with isolated angiitis of the CNS (2/6), Crohn-disease-associated CV (1/6), and reversible arterial vasoconstriction (RAV) of the CNS (1 migraine, 1 eclampsia and 1 toxic encephalopathy) (3/6). In all patients with RAV MRA showed a complete remission of the vascular alterations after treatment.

[Unilateral amaurosis as the only focal symptom caused by dissection of the common carotid artery].

Background: Central retinal artery occlusion with persistent amaurosis as the only focal symptom caused by dissection of the internal carotid artery has occasionally been reported. Central retinal artery occlusion due to a common carotid artery dissection has been diagnosed only very rarely.

Case Report: We describe a patient presenting with cervical pain, headache and unilateral amaurosis due to a thrombosis of the central retinal artery caused by a common carotid artery dissection, as demonstrated on MR imaging.

Rimmed vacuoles in facioscapulohumeral muscular dystrophy: a unique ultrastructural feature.

Rimmed vacuoles (RV) are a characteristic pathological feature in inclusion body myositis, but may also occur in other neuromuscular disorders, such as distal myopathies, oculopharyngeal myopathy, polymyositis, rigid spine syndrome, congenital myopathies, and some limb girdle muscular dystrophies, as well as in various neurogenic diseases. We describe a patient with RV in familial facioscapulohumeral muscular dystrophy (FSHD) associated with an FSHD-typical deletion on chromosome 4q35. Thus, FSHD should be included in the differential diagnosis of neuromuscular disorders with RV.

[The clinical spectrum of limb-girdle muscular dystrophies type 2I in cases of a mutation in the "fukutin-related- protein"-gene].

LGMD2I, linked to chromosome 19q13.3, is caused by mutations in the fukutin related protein (FKRP) gene. This myopathy has a variable clinical course with weakness and wasting of the shoulder girdle muscles and proximal extremities, calf hypertrophy, and elevated serum CK.

External ophthalmoplegia due to ocular myositis in a patient with ophthalmic herpes zoster.

External ocular muscle palsies in patients with ophthalmic zoster are traditionally interpreted as diseases of III, IV or VI cranial nerves. Orbital myositis associated with zoster ophthalmicus has been diagnosed only rarely. We describe a patient with ophthalmic zoster and external ophthalmoplegia due to ocular myositis demonstrated by MR imaging.

Magnetic resonance angiography in a patient with Crohn's disease associated cerebral vasculitis.

Vasculitits of the central nervous system (CNS) is a known complication of chronic inflammatory bowel diseases. We report the case of a female patient with Crohn's disease, developing vasculitis of the CNS with evidence of pathological vessel depiction on magnetic resonance angiography. To our knowledge this is the first case report regarding MRA documentation of vessel changes typical for angiitis in a patient with Crohn-associated vasculitis of the CNS.