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When brachialgia is also present, it takes several forms: a sharp pain in the pre- or postaxial border of the limb, extending to the elbow, wrist, or ngers; or a persistent dull ache in the forearm or wrist, sometimes with a burning sensation Discomfort may be elicited by coughing, Valsalva maneuver, or neck extension, or neck exion may induce electrical feelings down the spine (Lhermitte symptom) Rarely, the pain is referred substernally As to the sensory disorders (which may occasionally be absent), numbness, tingling, and prickling of the hands and soles of the feet and around the ankles are the most frequent complaints Some patients complain of numbness or paresthesias, most often in one or two digits, a part of the palm, or a longitudinal band along the forearm Slight clumsiness or weakness of a hand is another complaint.

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Lissencephaly Lissencephaly with cerebellar hypoplasia Lissencephaly (Miller-Dieker) or isolated lissencephaly X-linked lissencephaly with hypogonadism (Partington syndrome) Muscle-eye-brain disease Walker-Warburg Holoprosencephaly Double cortex Double cortex or X-linked lissencephaly Heterotopias Periventricular nodular heterotopia Tuberous sclerosis Tuberous sclerosis Fukuyama muscular dystrophy Schizencephaly Schizencephaly Microcephaly Microcephaly Microcephaly

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A feeling as if wearing gloves, swollen, or the hands coated with glue are common descriptions Several of our patients complained of paresthesias in the distal limbs and trunk for months before there was any indication of motor involvement In advanced cases there may be a vague sensory level at or just above the clavicles Impaired vibratory sensation and diminished position sense in the toes and feet (all indicative of a lesion of the posterior columns) as well as the Romberg sign are the most conspicuous sensory ndings This imparts a tabetic unsteadiness to the gait in advanced cases These sensory defects tend also to be asymmetrical (it is noteworthy that symmetric sensory symptoms and signs of identical type are seen with subacute combined degeneration due to B12 de ciency) Rarely, the sensorimotor pattern takes the form of a Brown-Sequard syndrome.

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Currently we prefer to distinguish these cases from typical GBS if only to lower expectations for the response to immune treatments and for the ultimate clinical recovery It should be reemphasized that the presence of electrically inexcitable nerves alone may be due to conduction block and give a misleading impression of axonal damage; the prognosis for cases with conduction block may be quite good The differential diagnosis includes critical illness polyneuropathy and the rarer entities of porphyric neuropathy and tick paralysis Laboratory Findings The most important laboratory aids are the electrodiagnostic studies and the CSF examination The CSF is under normal pressure and is acellular or contains only a few lymphocytes in all but 10 percent of patients; in the latter group, 10 to 50 cells (rarely more) per cubic millimeter, predominantly lymphocytes, may be found.

Less frequently, paresthesias and dysesthesias in the lower extremities and trunk may be the principal symptoms; even less often there are sensory complaints on the face, ostensibly corresponding to compression of the trigeminal sensory tract in the upper cervical cord The third part of the typical syndrome, spastic legs from a compressive myelopathy, most often presents as a complaint of weakness of a leg and slight unsteadiness of gait The whole leg or the quadricep feels stiff and heavy and gives out quickly after exercise Mobility of the ankle may be reduced, and the advancing toe of the shoe scrapes the oor On examination, hypertonicity is more evident than weakness, and the tendon re exes are increased (ankle jerks may not share in this change in the elderly).

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Although the patient may believe that only one leg is affected, it is commonly found that both plantar re exes are extensor, the one on the side of the stiffer leg being more clearly so Less often both legs are equally affected As the compression continues, walking becomes unsteady because of the addition of sensory ataxia The biceps and brachioradial re exes on one or both sides may be depressed, sometimes in association with an increase in the triceps and nger re exes The hand or forearm muscles, may un-.

The number of cells then decreases rapidly in a matter of 2 to 3 days; persistent pleocytosis suggests an alter-.

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