Brainstem Organization Of Supranuclear Ocular Motor Control

Supraoculomotor Nuclei

The planning and execution of ocular motor behavior involves the entire brain. Descending, centrally programmed saccadic, pursuit, and vergence commands, ascending vestibular commands, and cerebellar modulatory signals, all come together on ocular motoneurons. In considering the presumed profile of the system, we will start with immediate premotor structures responsible for carrying out all eye movement commands, then move on to cerebel-lar influences, and conclude with descending cortical...

General Management Of A Patient With Bacterial Meningitis

In patients with severe, life-threatening meningitis, the most important aspect of management is the immediate institution of empirical antibiotic therapy (Fig. 16.1 Table 16.1). We recommend that patients suspected of having bacterial meningitis, who present with a rapidly progressive course and severe alteration of mental status coma, receive an initial antibiotic dose immediately after the drawing of a single blood culture, prior to any other diagnostic procedures. In less acutely ill...

Mri

MRI sensitivity has been reported to vary between 50 and 100 .22 It approaches 100 in histologically confirmed cases, but there are no pathognomonic findings.6,22,23 Lesions suggestive of ischaemic and inflammatory changes, involving both the cortex and the white matter, have been reported. Some of these lesions are within known arterial territories,23-25 but this is not the rule, and it is also not uncommon for veins to be involved by the inflammatory...

Systemic Viral Infections And

Systemic infections, commonly viral, are recognized precipitants for CFS symptoms.17 The close proximity of poliovirus infection with epidemic CFS18 (often called 'atypical poliomyelitis' in the literature) also suggested a possible link with viral infection. The symptoms of CFS are also very similar to the postpolio fatigue symptoms (PPFS), the commonest sequel of poliomyelitis.19,20 There is a long list of Table 15.3 Neurological disorders causing symptomatic (secondary) fatigue not due to...

Table 172 Diagnostic criteria for sporadic CJD

II A Pyramidal or extrapyramidal features B Visual or cerebellar problems Definite confirmed Probable I + 2 of II + Ill A or llI B Possible I + 2 of II and duration < 2 years patients develop myoclonic seizures. These are initially driven by auditory (sometimes visual or tactile) stimuli (stimulus-sensitive myoclonus), but later become spontaneous, at a rate of about 1 Hz, which may or may not be symmetrical. Clinical criteria have been formulated for CJD. Some of these, not yet published...

Cerebrospinal fluid in PACNS

CSF is reported to be abnormal in 80-90 of pathologically documented cases of PACNS.6 An elevated protein level and lymphocytic pleocytosis are commonly found. The mean CSF protein was reported to be 177 mg (median 100 mg ) and the mean number of cells 77 mm3 (median 55 cells mm3).6 Increased IgG synthesis and the presence of oligoclonal bands are detected in some patients, but this finding has no diagnostic value. Normal CSF findings are accepted to have a high negative predictive value. CSF...

Human immunodeficiency virus

Human immunodeficiency virus (HIV) causes AIDS. The wide spectrum of neurological disease in AIDS patients results from direct HIV infection or from opportunistic infections secondary to the immuno-deficient state (Table 14.1). Clinical syndromes direct HIV infection ASEPTIC MENINGITIS After a prodromal viral illness, HIV infection may present with cranial nerve palsies,85 myelopathy,86 radiculopathy, peripheral neuropathy87 or spinal myoclonus.88 Most often, seroconversion is accompanied by...

Lumbosacral plexopathy LSP

Radiation-induced LSP (RLSP) is less common than RBP. A recent frequency estimate in a large-scale study of gynecologic tumors was less than 1 .40 Both prophylactic and therapeutic external-beam irradiation for various abdominal and pelvic neoplasms may cause RLSP when a portal includes the cauda equina or the lumbosacral plexus.41 Addition of intracavitary implants, as for gynecologic malignancies, concomitant chemotherapy and a pre-existing neurologic deficit may be contributing factors.9,42

Treatment Of IgM Paraproteinemic Neuropathy

The rationale for treating IgM paraproteinemic neuropathy is based on the assumption that the paraprotein plays an etiological role in the neuropathy. This evidence is particularly compelling in the cases associated with antibodies directed against MAG. Current treatment strategies include conventional immunosuppressive or immunomodulating regimens such as steroids, intravenous immunoglob-ulin, plasma exchange, alkylating agents or other chemotherapeutic drugs such as cyclophosphamide or...

Spon

Figure 51.4 Segments of a continuous record obtained in vitro from a long, intra-axonal impalement of a central axon at or near a site of demyelination induced by the injection of ethidium bromide into the dorsal column 14 days previously (resting potential -60 mV). The RPT for this axon was prolonged from 0.77 ms in its unaffected portion to 1.32 ms through the lesion. The record illustrates several electrophysiological properties of demyelinated axons. For all the records shown, the recording...

Plasmacytomas and multiple myeloma

Solitary plasmacytomas of bone (SPB) account for 5 of malignant plasma cell disorders.2 The spine is a frequent site of involvement, representing 25-60 of all SPB.1,3,4 A high incidence of SCC is reported in SPB, ranging between 43 and 71 of the cases.1,5,6 In contrast, in multiple myeloma, where spinal involvement is always present, the reported incidence of SCC varies between 10 and 16 .1,7,8 In many cases, SCC is the presenting manifestation of the disease, although the majority of spinal...

Osteosclerotic Myeloma

Osteosclerotic myeloma is a rare plasma cell dyscra-sia accounting for less than 3 of myeloma cases. Fully one-half of these cases are associated with a demyelinating polyneuropathy. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is present in a proportion of these cases. Improvement in the polyneuropathy has been shown following surgery or irradiation of solitary bone lesions.4-6 Responses in patients with multiple lesions have been less...

Vasculitis in focal and multifocal diabetic neuropathy

In a recent study on nerve lesions of the intermediate cutaneous nerve of the thigh and of the superficial peroneal nerve in patients with multifocal and proximal diabetic neuropathy, we found occlusion of epineural blood vessels associated with vasculitis and inflammatory in filtration in some patients.70 These findings come as a surprise in this setting. Clinically, the neuropathy was not accompanied by general signs or symptoms of inflammation. The lesions spared muscle blood vessels of the...

Info

Figure 51.3 Contour maps showing conduction along axons experimentally demyelinated with diphtheria toxin (a) or lysophosphatidyl choline (LPC) (b). Inward currents (indicating the presence of sodium channels) are shown by solid lines, and outward currents (indicating the presence of potassium channels) by dashed lines. The axons can conveniently be imagined to lie along the bottom axis, with nodes spaced approximately every 1 mm time progresses vertically. (a) shows conduction along two axons....

The Common Clinical Pattern

Paroxysmal manifestations in multiple sclerosis share several clinical characteristics. They correspond to positive symptoms or signs related to hyperactivity of the central nervous system. They occur suddenly, at any time or place. Their duration is short, a few seconds to 1 or 2 min. They often recur from a few times per day to several times an hour. Their semiological expression may vary considerably from one patient to another, but, as shown on the occasion of the frequent recurrences,...

The Neural Substrates Of Disorders Of Emotional Behaviour

How have theorists attempted to integrate the wealth of clinical reports on brain lesions and disorders of emotional behaviour Certainly, a comprehensive neuroanatomical and histological treatise on the brain bases of emotional behaviour is beyond the scope of this brief chapter. However, it is possible to paint a broad picture of current thinking as it has evolved over the course of the past decade and more.57,58 This thinking can be summarized succinctly as follows well-regulated, appropriate...

Treatment Strategies

There exist three primary treatment strategies for disorders of emotional behaviour pharmacological treatment, neuropsychological rehabilitation and ecological manipulation. Alas, few disorders of emotional behaviour have proven amenable to drug treatment. Three major exceptions are abulia, moria and pathological laughing and crying. Abulia may be responsive to low doses of stimulants such as methylphenidate,64 as well as dopamine agonists such as bromocriptine and lisuride.10,65 Moria may...

Update On Patent Foramen Ovale

Paradoxical embolism through a patent foramen ovale (PFO) is a known cause of embolic strokes and transient ischemic attacks in patients with stroke of uncertain etiology. Of considerable interest are recent reports on the variability of the detection of PFO when using different examination techniques. For example, the sensitivity of diagnosing PFO with both TCD and TEE is considerably higher when contrast media is injected into the femoral vein rather than into the antecubital vein, the...

Akathisia

This is a very common and early dose-related side-effect of neuroleptics. It may also occur after prolonged exposure to those drugs (tardive akathisia). It has also been described in patients with unmedicated encephalitic parkinsonism. Patients often complain of a feeling of inner tension in their limbs and body, causing them to shift from one position to another in attempts to relieve it. Any therapeutic strategy in individuals exposed to neuroleptics should include dose reductions, and...

Possible Explanations For The Paradoxical Effects Of Antiepileptic Drugs

Several facts are known about the paradoxical effects of AEDs. To begin with, they are not frequent in occurrence. Most information comes from case reports or small numbers of patients. Second, this phenomenon appears to occur more often in children with certain types of refractory epilepsy and with specific drugs. There are several possible and speculatory explanations for this phenomenon. An increase in seizures can occur with toxic levels of AEDs, such as that encountered in other types of...

Forearm Exercise Test

Five decades ago, McArdle designed an exercise protocol aimed at studying muscle glycolysis, which led him to define the first metabolic myopathy.8 He originally suggested that the defect was due to a defect in muscle glycogen breakdown,8 and later it was found that the primary defect in this condition was a deficiency of myophosphorylase.9,10 The disease, now called myophosphorylase deficiency or McArdle's disease, is the most common of the disorders of muscle carbohydrate metabolism, with an...

Usa

Department of Neurology Rehabilitation Centre CH-7317 Valens Switzerland Department of Neurology Ludwig-Maximilians-University Marchioninistrasse 15 Munich 81377 Germany 6 Brain Research Department of Neurology Carmel Medical Center 7 Michal Street Haifa 34362 Israel Department of Neurology and the Agnes Ginges Center for Human Neurogenetics Hadassah University Hospital Ein Kerem Jerusalem 91120 Israel Multiple Sclerosis Centre San Raffaele Hospital University of Milan Milan 20132 Section of...

Disorders Of Supranuclear Control Of Eye Movements

We will consider here major disorders of conjugate horizontal and vertical gaze and some vergence disorders. Owing to space limitations, we are forced to leave out fixation disorders, such as most forms of nystagmus and saccadic intrusions for them, the reader is referred to standard texts.1,44 Damage to the abducens nucleus results in a rare but fairly characteristic pattern of a 'nuclear VI' inability to activate the ipsilateral lateral rectus and contralateral medial rectus for all types of...

Expression Of Demyelination In Eae In The Context Of Antibodies

Increasing evidence correlates the presence and levels of anti-MOG antibodies with demyelination in EAE. This was first suggested by the demonstration that the demyelinating activity of chronic EAE sera assayed in vivo by intrathecal injection of normal rats is proportional to the anti-MOG antibody titer of the serum.45 In guinea pigs with chronic EAE, antibodies against MOG are not always detected in sera but are always present in acid extract of CNS tissue, with levels correlating with...

References

The cerebrospinal fluid in multiple sclerosis. In Hallpike JF, Adams CW, Tourtellotte WW, eds. Multiple Sclerosis. London Chapman and Hall, 1983 275-358. 2. Kerlero de Rosbo N, Bernard C. Multiple sclerosis brain immunoglobulins stimulate myelin basic protein degradation in human myelin a new cause for demyelina-tion. J Neurochem 1989 53 513-518. 3. Prineas JW, Graham JS. Multiple sclerosis capping of surface immunoglobulin G on macrophages engaged in myelin...

General Guidelines

Several issues should be considered before prescribing a medication for a recently diagnosed PD patient, as follows. Does a recently diagnosed patient need medications and, if so, what should be the goal of treatment Since no definite neuroprotective agent is yet available, there is no rush to begin pharmacotherapy. Physical exercises should be encouraged if the patient does not experience significant functional disability. The therapeutic strategy should be discussed with the patient as well...

Therapeutic Aminoglycoside Ototoxicity

'Functional labyrinthectomy' with ototoxic aminogly-cosides (gentamicin or streptomycin), proposed by Schuknecht in 1957,26 was first tried in Europe with 8-24 mg gentamicin sulfate (Refobacin) instilled daily through a plastic tube inserted behind the anulus via the transmeatal approach.27,28 At that time it was thought that it is possible to selectively damage the dark cells of the secretory epithelium (and thereby improve endolymphatic hydrops) before significantly affecting vestibular and...

Thrombolytic Therapy

Arterial occlusion in the appropriate distribution of brain infarction has been observed angiographically in 75-80 of patients within 8 h of onset of symptoms.17-19 Spontaneous clot lysis, probably resulting from activation of an endogenous plasminogen activator, occurs from the first to the seventh day after onset of stroke in 14-73 of patients,17,19,20 but is usually not accompanied by clinical improvement.17 Trials of intravenous thrombolysis with different designs carried out prior to 1995...

Martin Dunitz

2002 Martin Dunitz Ltd, a member of the Taylor & Francis Group First published in the United Kingdom in 2002 by Martin Dunitz Ltd, The Livery House, 7-9 Pratt Street, London NW1 0AE Tel +44 (0) 20 7482 2202 Fax +44 (0) 20 7267 0159 E-mail info dunitz.co.uk Website http www.dunitz.co.uk This edition published in the Taylor & Francis e-Library, 2004. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any...

Pre And Postherpetic Neuralgia

Although the rash of zoster and pain typically occur within days of each other, there are reports of dermatomal distribution pain that precedes rash by 7-100 days (pre-herpetic neuralgia).24 PHN, pain that persists for more than 4-6 weeks after zoster, is common, and affects more than 40 of zoster patients over age 60.25-27 Double-blinded placebo-controlled trials have shown that patients obtain relief with topical aspirin in chloroform,28 gabapentin,29 tricyclic antidepressants and...

Lymphomatous Meninigitis

CNS lymphoma may present as lymphomatous meningitis, a condition characterized by headache, fever, stiff neck and mental status changes with cranial or spinal nerve palsies. CSF white blood cell count, protein and glucose can be normal or elevated. CSF cytological analysis may reveal malignant cells. Brain imaging reveals atrophy with or without meningeal enhancement. The mean survival from the time of diagnosis of lymphomatous meningitis is only 5 weeks. At this stage, treatment is not usually...

Cryptococcus

One of the most common opportunistic infections and the most common fungal infection in AIDS patients is cryptococcal meningitis. Infection does not usually occur until the CD4 count is 46 cells mm3 or less. Mortality approaches 30 within 3 months after diagnosis.150 CSF India ink staining, CSF and serum cultures and Cryptococcus antigen studies all help in diagnosis. Optimal treatment is controversial, because of high failure rates with current protocols. A reasonable approach is intravenous...

Varicellazoster virus

VZV causes chickenpox (varicella), becomes latent in cranial and dorsal root ganglia, and may reactivate decades later to produce shingles (zoster). Reactivation occurs most commonly in elderly and immuno-compromised individuals. Although zoster rash is usually temporally associated with neurological disease, all of the above conditions, most notably meningoencephalitis, cranial neuropathies and myelitis, may occur without antecedent rash.14 Herpes zoster consists of dermatomal distribution...

Matrix Metalloproteinases In Cidp

Matrix metalloproteinases (MMPs) are proteolytic enzymes that are involved in the remodeling of the extracellular matrix (ECM) in a variety of physiological and pathological processes.74 The MMP family consists of at least 20 members. They are categorized into the collagenase, gelatinase, stromelysin and membrane-type subfamilies. Because MMPs can catalyze the degradation of all the protein constituents of the ECM, their activities are kept under tight control. MMPs are regulated in three ways...

Disorders Of Overt Emotional Behaviour

Abulia is characterized by loss of interest in and motivation to engage in purposeful behaviour. The abulic patient is passive, initiates little, lacks spontaneity, and finds no pleasure in former pursuits. However, affective and cognitive components of major depression, such as dysphoria, loss of optimism, guilt feelings and suicidal ideation, are lacking. Initial symptom onset follows brain disease or trauma. Abulia is most commonly seen in left hemispheric lesions, particularly of the...

Myoclonus

This is a sudden, rapid, irregular, lightning-like movement produced by an abrupt and brief muscular contraction (positive myoclonus) or inhibition (negative myoclonus) like asterixis. Myoclonus may be classified as focal, multifocal, segmental or generalized, and, according to the site of origin, as cortical, brain stem (reticular) or spinal. Cortical back-averaging evoked potentials and polyelectromyography have permitted the physiological subclassification of myoclonus into cortical,...

HIBM1 dominant forms

At least six families have been reported.4-5 In all of them, patients have adult-onset (4th or 5th decade) proximal and distal muscle weakness, either in the upper or in the lower limbs. The quadriceps is affected. Serum creatine kinase (CK) is normal or mildly elevated. Brain magnetic resonance imaging (MRI) was not reported. B. A single French-Canadian family, with a fascio-scapulohumeral dystrophy-like HIBM starting distally in hand muscles at early childhood in the mother and proximally...

Neurophysiology of inflammatory demyelinating disease

Diseases such as multiple sclerosis (MS) and Guillain-Barre syndrome (GBS) result in inflammatory demyelinating lesions within the central and peripheral nervous systems (CNS, PNS) respectively. The lesions cause a range of conduction abnormalities and these lead directly to the symptoms expressed. The nature of the particular symptoms expressed depends upon the pathway affected by the lesion. RELAPSE AXONAL CONDUCTION BLOCK Demyelination Perhaps the most prominent conduction deficit in...

Management of paroxysmal symptoms in multiple sclerosis

Christian Confavreux, Fran oise Bouhour and Sandra Vukusic The first anecdotal reports on paroxysmal symptoms or signs in multiple sclerosis date back to the last century. The first comprehensive review was made by McAlpine in 1972 in his classical monograph1 devoted to multiple sclerosis with the collaboration of Lumsden and Acheson. Several other authors should also be mentioned for their pioneer work in the field. The description of tonic seizures is due to Matthews,2 paroxysmal dysarthria...

Arboviruses

The arthropod-borne (arbo) viruses include toga-viruses, bunyaviruses and reoviruses. Transmission from infected animals to humans takes place in the summer or autumn through mosquito or tick vectors. Myalgia is common. Tremor and seizures are frequent features of the encephalitis. Inoculation of the patient's CSF into young mice may produce encephalitis. Antiviral antibody may also be detected in the CSF. The presence of serum IgM or a rising titer of IgG against the particular virus is strong...

Nervous system vasculitis secondary to infections and related conditions

Infections due to viruses, bacteria, fungi, and protozoa may all cause vascular inflammation within the nervous system.6,41 These organisms are also associated with systemic vasculitis, and it is not uncommon for them to induce either focal or diffuse cerebral vasculitis and present with neurological manifestations in the absence of clinical evidence of systemic involvement. In many instances, the responsible organism may be angio-invasive, but in some the vascular inflammation is related to...

Disorders Of Emotional Experience And Communication

Alexithymia is a cognitive-affective disturbance of the ability to recognize and describe one's own feelings and emotional states. It can occur after brain injury or disease, although it is also well described in the psychiatric literature.42 Alexithymics have a poverty of inner fantasy life, constricted emotional activity, and few if any expressive facial movements, and sometimes have rigid posture as well.43 It has been reported in cases of agenesis of the corpus callosum44 as well as other...

Wegeners Granulomatosis

In Wegener's granulomatosis, which is characterized by granulomatous vasculitis of upper and lower respiratory tract with or without glomerulonephritis, peripheral neuropathy occurs in 25 of the patients.32 In this group, smaller vessels are affected and, in our experience, Wegener's granulomatosis is much less frequently the cause of a peripheral neuropathy than in the previous groups. The neurological manifestations of Wegener's granulomatosis have been reviewed in a large retrospective study...

Human Antibodies Promote Remyelination In Tmevinfected Mice

Antibody treatment is currently being explored as a therapy for human demyelinating disease. Intravenous immunoglobulin IVIg has been used successfully to treat a variety of autoimmune neurologic diseases, including Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuro-pathy, multifocal motor neuropathy, polymyositis, and myasthenia gravis.26 Clinical studies in MS indicate that IVIg may be effective in stabilizing the course of the disease.27 Given the demonstrated ability of a...

Zoster Sine Herpete

Prolonged, burning radicular pain without rash can be caused by VZV. Proof was evident in two subjects with long-standing radicular pain without rash in which PCR demonstrated VZV DNA in the CSF. Both patients were successfully treated with intravenous acyclovir, 15 mg kg three times daily for 14 Ataxia, nystagmus and nausea with movement may be associated with acute chickenpox and develop either before44 or after45 rash. In one subject,44 the EEG showed bilateral slowing with sharp bursts of...

Mononeuritis Multiplex

In late HIV disease CD4 count below 50 cells mm3 , patients may develop extensive mononeuritis with rapid progression49 and, without treatment, may die within 3 months.50 EMG shows multiple axonal mononeuropathies beginning distally and spreading proximally. Biopsy may show necrotizing vasculitis, cryglobulinemia, axonal and demyelinating lesions, and CMV inclusions.51 Gancyclovir may stabilize50 or reverse49 the deficits in CMV-induced mononeuro-pathy multiplex, and plasmapheresis provided...

Premorbid Personality

Repeated attempts have been made, in retrospective investigations of patients already affected, to characterize a premorbid personality, and to determine characteristics of personality which dispose to the disease. Such investigations8 claimed that the premorbid personality of MS patients should be characterized by hysterical aspects, as notes on 'hysteria' are frequently found in case reports of MS patients. In fact, MS may manifest itself in earlier phases of the disease by various symptoms...