Isaacs' syndrome associated with chronic inflammatory demyelinating polyneuropathy.

We report the first case of Isaacs' syndrome in which an inflammatory demyelinating neuropathy was documented histologically. For 9 months, the patient developed slowly progressive weakness, muscle spasms and stiffness, fasciculations, and myokymia in the arms, which were unmodified by sleep. Nerve conduction studies showed multifocal motor conduction block, abnormal dispersion phenomenon, and abnormal sensory and mixed nerve conduction. Needle electromyogram showed continuous motor unit potentials at rest with bursts of rapid-firing discharges which were unaffected by spinal anesthesia but diminished by peripheral nerve block and completely abolished by local curarization. Sural nerve biopsy demonstrated an inflammatory demyelinating neuropathy. Muscle cramping, twitching, and stiffness responded to phenytoin. The patient's weakness gradually responded to prednisone and azathioprine. Over a 17-year period, the patient had three relapses which were well controlled with prednisone and azathioprine. At this time, the patient is symptom-free without any medication.

Vasculitic neuropathy mimicking Guillain-Barré syndrome.

Vasculitic neuropathy classically manifests as a subacute mononeuritis multiplex or polyneuropathy. Patients with a Guillain-Barré syndrome (GBS)-like presentation are usually not considered to have an underlying vasculitis. We describe 2 patients with rapidly progressive areflexic paralysis that was initially diagnosed as GBS, in whom vasculitic neuropathy was found histologically.

"Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy.

The clinical electrophysiological and histological features of 10 cases of "chronic sensory demyelinating neuropathy" (CSDN) are reported. This entity is characterised by: 1) subacute or chronic progression; 2) pure sensory neuropathy; 3) high spinal fluid protein in the majority of cases; 4) electrophysiological evidence of demyelination affecting motor as well as sensory nerve fibres; 5) demyelination on sural nerve biopsy and 6) good response to immunotherapy in progressive phase. It is believed that this entity represents chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as pure sensory neuropathy.

Electrophysiological spectrum of inclusion body myositis.

We present electrodiagnostic data on 30 patients with inclusion body myositis (IBM) in order to better delineate its electrophysiological features. Comprehensive electromyography (EMG) and nerve conduction studies (NCS) were performed in all cases. Twelve patients had single fiber electromyography (SFEMG). EMG showed abundant short-small motor unit potentials (MUP) with fibrillations and positive sharp waves in 56.6% of patients, and a mixed pattern of large and small MUP in 36.7%. In 6.7%, only "neurogenic" features were seen. NCS were slow in 33.3%. SFEMG revealed a mildly abnormal jitter and a slightly increased fiber density. IBM demonstrates a heterogeneous EMG profile. A pattern of large and small MUP is highly suggestive of IBM but is seen in only about one third of cases.

Tomaculous neuropathy presenting as acute recurrent polyneuropathy.

Tomaculous neuropathy (TN) is classically associated with the inherited, recurrent focal neuropathies. We report a case of TN manifesting as an acute recurrent polyneuropathy. A 28-year-old woman had 2 episodes of acute, ascending, symmetrical sensorimotor deficit. Teased nerve-fiber preparation confirmed the presence of TN. Extensive investigations failed to reveal other cause for her symptoms. We believe that this case is unique and broadens the clinical spectrum of TN.

Asymptomatic hyper-CK-emia: an electrophysiologic and histopathologic study.

Since the popularization of routine creatine kinase (CK) measurement, an increasing number of patients with unexplained CK elevation ("asymptomatic hyper-CK-emia") are being identified. We studied 19 patients with persistent CK elevation of unknown etiology with electromyography (EMG) and muscle biopsy. Needle EMG was abnormal in 14 patients. Muscle biopsy was positive in all individuals with abnormal EMG and in one patient with normal EMG. Diagnoses included polymyositis in five patients, morphologically nonspecific myopathy in three, mitochondrial myopathy in two, and sarcoid myopathy, central core disease, multicore disease, inclusion body myopathy, and McArdle's disease in one case, respectively. Five patients with abnormal biopsies developed weakness within 1 year of presentation. We conclude that persistent asymptomatic CK elevation represents mild or early myopathy in a majority of cases.

The syndrome of cerebral infarction following herpes zoster ophthalmicus.

Delayed contralateral hemiparesis following herpes zoster (HZ) ophthalmicus is an unusual but distinct clinical entity, presumably caused by HZ-induced arteritis with subsequent cerebral infarction. We report a case showing typical clinical and angiographic findings.

Functional classification of undernourished populations in the Republic of El Salvador. Methodological development.

Typical government nutrition programmes usually lack precise information on the size of groups that could benefit from interventions, on the different types of potential beneficiaries and their location, and on socio-economic and cultural characteristics through which they could readily be identified. In the functional classification approach to the definition of nutritional problems, detailed information on human behaviour and social constraints is collected at family and community levels. The data are then interpreted in general terms in order to understand how these factors contribute to inadequate levels of nutrition within larger groups. This new approach should enable more effective measures for reducing the numbers of those living under conditions of deprivation to be presented to planners and decision makers.

Nutrition and national development planning.

We have argued that development strategies aimed at the reduction of all forms of deprivation, according to an explict statement of values and priorities, are necessary for the integration of nutrition planning into overall national development planning. We do not regard this as likely to lead to the neglect of the key issues of investment and production. Instead, we argue that consideration of investment and production strategies should be explicitly directed to their purpose, the reduction of deprivation, rather than, as in the past, treated as the necessary means to that purpose. Thus investment and production strategies would be aimed directly at relieving deprivation including, especially, nutritional deprivation, and their impact in this respect will need to be explicitly predicted and evaluated. We have argued that problems and potentials differ greatly at the area level and that planning must be an iterative process in which national and area-level strategies and programmes are brought to consistency through successive cycles of adjustment and reappraisal. A major role in planning, especially in detailed design and implementation, is assigned to area level. Planning must proceed from an identification of the deprivations under attack to the identification of intervention measures. Elsewhere we have argued the case for the 'functional classification" of malnutrition and the use of "typical profiles" in the understanding of "the ecology and etiology of malnutition". Where planning accepts the need for an integrated approach to the attack on all forms of deprivation, these analyses of nutritional deprivation would from part of a more comprehensive analysis of general deprivation. The existence of "functional classification" and "typical profiles" analysis with respect to malnutrition would provide an excellent base from which a more comprehensive view might be developed. The factors affecting the evolution of nutritional - and other - deprivation problems need to be understood in order that we can identify points in the system where intervention may control theri emergence. In any situation there will be alternative intervention points bearing either directly on the alleviation of symptoms (malnutrition) or, more or less indirectly, on the control of its causes. In general, mixed strategies will be called for. At this stage in our understanding no generalizations are possible about the nature of strategy choices. Our choice of intervention measures must be based upon an understanding of the overall system as it generates malnutrition, and of which forces exert the most powerful effects. One important element of this analysis will be an understanding of the behaviour of the malnourished. However, conventional planning approaches, in which ministries and departments concern themselves only with problems and measures which conform to the definition of their own spheres of responsibility, have failed to define these choices effectively...