[Postpartum footdrop due to compression of the lumbosacral trunk]. / Geburtstraumatische Fussheberpareseinfolge einer pelvinen Nervenschädigung.

We report on a rare peripartal neuropathy of the leg caused by pro longed difficult labor in which forceps were used or caesarean sec tion was performed. Immediately after delivery, the 2 patients corn plained of a unilateral footdrop and numbness in the leg. The foot drop was most likely due to a compression of the lumbosacral trunl exposed to the fetal head. This trunk contains fibres from the lumba roots L4 and L5 and connects the lumbar with the sacral plexus. The outcome was favourable in both patients. If subsequent pregnancies occur, caesarean section may be indicated.

[Vasculitis and mononeuritis multiplex]. / Vaskulitis und Mononeuritis multiplex.

In 1991, a 67 year old patient, suffering from an adenocarcinoma of the colon, had to undergo hemicolectomy. In the five following years, isolated metastases developed in the liver and have been surgically removed. In 1997, acute mononeuritis multiplex appeared and nerve biopsy revealed vasculitis. There were no signs of inflammatory rheumatic illness, collagenosis, cryglobulinemia, infection or hypersensitivity to medication. We have therefore postulated a relation between the vasculitis and the malignancy. Tumorous relapse could however not be detected at that time. Under immunosuppressive therapy, the neurological deficiencies receded partially, though the carcinoma of the colon reappeared with systemic metastases in the wake of the vasculitis. Diagnostic measures and the relationship between malignancy and vasculitis are discussed.

[Descending paralysis caused by wound botulism. A case report]. / Deszendierende Lähmung durch Wundbotulismus. Eine Falldarstellung.

We report on the history and clinical findings of an injecting drug abuser in the Canton of Zurich who presented with multiple deep abscesses in the arms and legs. A diagnosis of wound botulism was made based on his clinical presentation with a rapidly progressing descending paralysis starting at the cranial nerves, a neuromuscular junction disorder on neurophysiologic testing, and normal findings on lumbar puncture. Several cases of wound botulism have occurred in i.v. drug abuse in Switzerland since 1997. We suspect subcutaneous injections of contaminated heroin containing Clostridium spores as sites of entry. Wound botulism caused by Clostridium botulinum is a rare cause of rapidly progressing, generalized, flaccid paralysis and should be considered in patients with a history of i.v. drug abuse presenting with descending paralysis.

[Postpartum footdrop]. / Postpartaler Fallfuss.

We report on a rare peripartal neuropathy of the leg caused by prolonged difficult labour. Immediately after delivery two patients complained of unilateral footdrop and numbness in the leg. The footdrop was most probably due to compression of the lumbosacral trunk exposed to the foetal head. This trunk contains fibres from lumbar roots L4 and L5 and connects the lumbar with the sacral plexus. The outcome was favourable in both patients. If subsequent pregnancies occur, caesarean section may be indicated.

Canal and otolith afferent activity underlying eye velocity responses to pitching while rotating.

Pitching the head while rotating (PWR) combines periodic activation of the semicircular canals and the otoliths to generate pitch and roll eye deviations and continuous horizontal nystagmus. Monkeys were tested after individual pairs of semicircular canals were plugged and single units were recorded in the vestibular nerve while the animals were sinusoidally pitched 20-40 deg about a spatial horizontal axis with 5- and 16-s periods and simultaneously rotated about a spatial vertical axis at 30-120 deg/s. As previously shown, the steady-state horizontal response disappeared after plugging the vertical semicircular canals, but was maintained when the lateral canals were plugged. When the left anterior and right posterior canal (LARP) pair was left intact, the steady-state response depended on the axis about which the pitching took place. When the axis was normal to the LARP plane, there was no steady-state response. When the pitching axis was perpendicular to the LARP normal, the response was maximal. Firing rates of otolith units were approximately in phase with pitch position, and the addition of rotation about a vertical axis did not change the response. Lateral canal units did not have a steady-state modulation during pitch or constant velocity rotation. During PWR, they oscillated at twice the pitch frequency. This corresponded to the frequency at which the canal was maximally activated as it aligned with the plane of rotation. The amplitude of modulation increased proportionally to rotational velocity, but the phase remained the same. These characteristics were unchanged during roll while rotating (RWR), which induces little continuous nystagmus. Anterior and posterior canal units were maximally excited near pitch-velocity maxima and minima, respectively, during pure pitching. During PWR, however, the phases of both components simultaneously shifted toward each other and toward being in phase with otolith units. The peak excitation tended toward a forward-pitch position when the rotation was to the ipsilateral side, and toward a backward pitch position when the rotation was to the contralateral side. With 120-deg/s rotation during a 16-s pitch period, the phase difference between anterior and posterior canal units was as small as 17 deg. These data support the postulate that the correlation between vertical canal and otolith units is the critical factor in generating continuous unidirectional horizontal nystagmus during PWR.

[Benign postural vertigo and nystagmus of the horizontal semicircular canal]. / Der gutartige Lagerungsschwindel und -nystagmus des horizontalen Bogenganges.

Benign paroxysmal vertigo and nystagmus are induced not only by the posterior but also by the horizontal semicircular canal. Benign positional nystagmus of the horizontal canal is more often observed than was previously thought. In 10 patients we analyzed the characteristics and the variability of nystagmus which accompanies positional vertigo of the horizontal canal. There are two forms of nystagmus: primary-geotropic, most often paroxysmal nystagmus (7 patients), and primary-apogeotropic, non-paroxysmal nystagmus (3 patients). Interestingly, in 2 patients with the primary-apogeotropic form the nystagmus converted during the examination into the primary-geotropic form. The reverse was not observed. We discuss the possible pathophysiological mechanisms which could be relevant for provoking manoeuvres.

[Adult form of GM2-gangliosidosis: a man and 2 sisters with hexosaminidase-A and -B deficiency (Sandhoff disease) and literature review]. / Adulte Form der GM2-Gangliosidose: drei Geschwister mit Hexosaminidase-A- und -B-Mangel (Morbus Sandhoff) und Literaturübersicht.

Three adult siblings had atypical progressive spinal muscular atrophy of the limb-girdle type, predominantly sensory polyneuropathy and cerebellar ataxia. Hexosaminidase A and B activity was profoundly decreased in serum, leukocytes and cultured fibroblasts. GM2-gangliosidosis, variant O (Sandhoff disease) was diagnosed. Mechano-allodynia was the presenting symptom in two of the patients. After 50 years of disease evolution, the patients led an independent life and were intellectually normal. The literature on the adult form of GM2-gangliosidosis is reviewed.

[Benign paroxysmal postural vertigo: basis and its therapy in clinical practice]. / Der gutartige paroxysmale Lagerungsschwindel: Grundlagen und seine Therapie in der Praxis.

The theory of canalolithiasis assumes that benign paroxysmal vertigo and nystagmus is due to particles within the semicircular canal which move freely in the endolymph under gravitational forces, thereby deflecting its cupula. According to this theory the particles could be removed from the posterior canal by manoeuvres applying a succession of different head positions. We describe the pathophysiological mechanism of benign paroxysmal vertigo and our experience with one of these manoeuvres, the Semont manoeuvre. The success rate is impressive and the therapy is highly rewarding in a vertigo syndrome which can cause disability in the patients.

[Acute vestibular syndrome in cerebellar infarct of the posterior inferior cerebellar artery (PICA infarct)]. / Akutes vestibuläres Syndrom beim Kleinhirninfarkt der Arteria cerebellaris posterior inferior (PICA-Infarkt).

The differential diagnosis of an acute peripheral vestibulopathy ("vestibular neuritis") and of an ischemic lesion in the cerebellar territory of the posterior-inferior cerebellar artery (PICA) is important. Both present with acute vertigo, vomiting, spontaneous nystagmus and difficulties in walking. We analyze the clinical, oculographic and NMR findings as well as the outcome in 10 patients with an ischemic stroke in the cerebellar territory of the PICA. On clinical grounds alone, it is not always possible to attribute the acute vestibular syndrome to a peripheral vestibular lesion or to a PICA insult. In our experience an important feature of a PICA insult is a discrepancy between the amount of vertigo, the severity of the walking difficulties, and the often weak and rapidly resolving spontaneous nystagmus. In contrast to a peripheral vestibulopathy, the vestibuloocular reflex is only minimally or not decreased in PICA insults, and gain asymmetries are accounted for by the presence of spontaneous nystagmus. However, only neuroimaging can finally differentiate between the two entities.

Early and severe sensory loss in three adult siblings with hexosaminidase A and B deficiency (Sandhoff disease).

Three siblings in their sixth and seventh decade with hexosaminidase A and B deficiency (adult form of GM2-gangliosidosis, variant O) developed early and severe sensory loss in addition to chronic motor neuron disease and cerebellar ataxia. Prominent mechanoallodynia was a manifesting symptom in two siblings. It is suggested that sensory deficits are due to a central-peripheral dying back axonopathy. The early and dominant sensory disturbances extend the clinical range of GM2-gangliosidosis.

[Differential diagnostic aspects of progressive spastic paraplegia in adults with emphasis on neurometabolic diseases]. / Differentialdiagnostische Aspekte der progredienten spastischen Paraparese beim Erwachsenen mit Betonung neurometabolischer Erkrankungen.

The differential diagnosis of a progressive spastic paraparesis in the young adult is broad and includes rare neuro-metabolic diseases like cerebro-tendinous xanthomatosis, adrenomyeloneuropathy and hypovitaminosis. Their clinical presentation as well as the result of paraclinical examinations can be similar to those of multiple sclerosis. The early recognition of these diseases is important, because a dietary regimen may reduce the severity and progression of symptoms and signs and genetic counselling can be important. The relevant biochemical examinations for their detection are discussed. These neuro-metabolic diseases have to be differentiated from other neuro-degenerative diseases like amyotrophic lateral sclerosis and hereditary spastic paraplegias.

Eye movement disorders.

The past year's literature on eye movements is characterized by two trends: first by the examination of eye movement organization in the three planes of eye rotation in normal subjects (there is yet no publication about abnormalities of this three-dimensional organization in patients) and second by the increasing awareness of clinicians that the characteristics of saccades are highly task-dependent, and that the saccadic performance of patients must be tested under various conditions. Several papers try to establish a correlation between the site of a lesion and abnormalities of specific parameters of task-dependent saccades. For a better understanding, the different experimental tasks for the elicitation of saccades are explained in the text.

[Spondylogenic cervical myelopathy: conservative and surgical therapy]. / Die spondylogene zervikale Myelopathie: konservative und operative Therapie.

The symptoms and signs of spondylotic cervical myelopathy (SCM) in the elderly are variable, but an ataxic-spastic gait disturbance combined with tingling in the finger tips and clumsy hands are often encountered. SCM is due to degenerative changes of discs and osseous and ligamentous structures with compression of the spinal cord. The symptoms, signs, surgical and conservative treatment, and outcome were retrospectively analyzed in 36 patients. Most of the patients undergoing surgery (n = 22) improved their clinical score and maintained this improvement for the period of observation; the non-operated patients (n = 14) had a very slow progression. Diagnostic difficulties and problems of the surgical and conservative approaches are discussed.

[Differential diagnosis of chronic gait disorders of neurologic origin in old age]. / Die Differentialdiagnose neurologische bedingter chronischer Gangstörungen im Alter.

The differential diagnosis of chronic gait difficulties with falls in the elderly, due to neurological diseases, is discussed. The spectrum is quite wide and encompasses central and peripheral pathologies of the nervous system. For centrally induced gait difficulties auxiliary examinations correspond to those used in the evaluation of dementia.

[Treatable diseases of the nervous system with cataract formation]. / Behandelbare Erkrankungen des Nervensystems mit Kataraktbildung.

The detection of a cataract in combination with a neurological deficit may provide the physician with important diagnostic help. But a minority of underlying diseases (angiokeratoma corporis diffusum, cerebrotendinous xanthomatosis, diabetes mellitus, galactosemia, hypocalcemia, Refsum's disease, Wilson's disease; Charles Bonnet syndrome; relapsing Perichondritis; adverse effects of medication and intoxications) can be treated causally. Therefore they are summed up and discussed in this paper.