Management of voltage-gated potassium channel antibody disorders.

Syndromes from antibodies to voltage-gated potassium channels include neuromyotonia (NMT), limbic encephalitis (LE) and Morvan syndrome (MVS). There are distinct clinical features for NMT (cramps, stiffness, fasciculations, myokymia, hyperhidrosis; afterdischarges and continuous motor activity on electromyogram), LE (encephalopathy with seizures, deficient recent memory; hyponatremia, temporal lobe magnetic resonance imaging and electroencephalographic abnormalities) and MVS (NMT plus hyperhidrosis, dysautonomia, encephalopathy, severe insomnia, and sleep disorders). There may be associated myasthenia gravis or thymoma, and rarely lung cancer (small cell or adenocarcinoma), mandating that chest imaging be part of the evaluation.

Arm restlessness as the initial symptom in restless legs syndrome.

Background: Upper extremity symptoms can develop in restless legs syndrome, but are rarely the initial symptom.

Objectives: To report a case of restless legs syndrome with restless arms as the initial symptom, and to review the literature.

Design: Case report and literature review.

Transient visuospatial disorder from angiographic contrast.

Background: The blood-brain barrier may be permeable under the clinical settings of uncontrolled hypertension, renal insufficiency, immunosuppressive drugs, and intravascular radiographic contrast. Some reversible neurological complications after angiography are caused by cortical penetration of contrast media detected on brain computed tomographic (CT) scans.

Objectives: To describe the first report of a transient visuospatial disorder having elements of Balint syndrome, and caused by angiographic contrast penetration of the bilateral parieto-occipital cortex; and to review cases published between 1980 and 2001 of cortical contrast penetration, documented by CT.

Segmental zoster paresis of limbs.

Segmental zoster paresis (SZP) is the focal, asymmetrical neurogenic weakness which may occur in a limb affected by cutaneous zoster. We have summarized the features of this syndrome, based on a retrospective review of 8 personal and 96 published cases. Limb SZP becomes apparent in at least 3-5% of patients with cutaneous zoster, who are usually over the age of sixty and weak proximally (C5,6,7 or L2,3,4 innervated muscles).

Quantitative sensory threshold testing in patients with multiple sclerosis.

Cutaneous thresholds for vibratory and thermal stimuli were quantitated at the index finger and great toe of twelve patients with definite multiple sclerosis. Nine of 12 (75%) patients had abnormalities to either modality, especially at the great toe, where 48% of vibratory and 17% of thermal thresholds were abnormal. Of ten patients undergoing somatosensory evoked potential (SSEP) testing, 8 (80%) had abnormal median and 9 (90%) had abnormal tibial studies.

Renal causes of elevated sedimentation rate in suspected temporal arteritis.

The erythrocyte sedimentation rate (ESR) is a frequently used but nonspecific indicator of inflammation or infection. Clinicians often check an ESR in patients with symptoms of headache, facial or jaw pain, and visual loss, as an aid in the diagnosis of temporal arteritis. We present two patients with these complaints, who did not have temporal arteritis, nor any other inflammatory condition or infection, but had ESRs near or above 100 mm/h, leading to diagnostic confusion.

Complex partial seizure-induced transient MR enhancement.

A 58-year-old man with complex partial seizures had transient symmetrical MR enhancement in the anterior mesiotemporal cortex bilaterally, correlated with the sites of seizure focus on electroencephalography. Preinfused T1-weighted and T2-weighted sequences showed no abnormality. After adequate control of seizures with medication, repeat MR was normal and the prior enhancement was no longer seen.

Systemic lupus erythematosus with central nervous system involvement.

Real or suspected brain involvement occurs in the majority of patients with systemic lupus erythematosus. The clinical manifestations are myriad and are accounted for by diverse pathogenic mechanisms. Purely psychological disturbances and psychiatric syndromes with organic components account for the majority of cases.

Olivopontocerebellar atrophy presenting with hemiparkinsonian ocular motor signs.

A patient with olivopontocerebellar atrophy presented with an asymmetric parkinsonian syndrome consisting of right limb rigidity, bradykinesia, masked facies, and a hypophonic, monotonous voice. Right limb clumsiness suggested corticospinal tract involvement. Gait was slightly wide based, but no other cerebellar or brainstem signs were present.

Cardiac transplantation in female Emery-Dreifuss muscular dystrophy.

A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities. Myopathy and heart disease were not clinically evident in her family, although three relatives had unexplained shortened Achilles tendons without weakness.

Aging and quantitative sensory thresholds.

Cutaneous thresholds for vibration and thermal sensitivity were quantitated at the index finger and great toe in 54 subjects without obvious neurological disease. Twelve patients over sixty years of age also consented to standard nerve conduction studies which proved normal. Vibration thresholds progressively increased with age to a significant degree at the finger (p less than 0.

Quantitative sensory thresholds in carpal tunnel syndrome.

Thresholds for vibration and temperature sensation were quantitated at the second and fifth digits of 23 patients (28 hands) with symptomatic, electromyographically confirmed, carpal tunnel syndrome. Thresholds were determined in 23 age-matched controls. Abnormal sensory threshold data confined to the second digit, apparently related to carpal tunnel syndrome, was found in only 3 (11%) of 28 symptomatic hands.

Brain metastases from undiagnosed systemic neoplasms.

Fifty-six patients presenting with symptomatic brain metastasis but undiagnosed primary neoplasm were retrospectively studied. Metastases were almost equally solitary (57%) as multiple (43%), and 30% were cerebellar. Cerebral metastases were most often parietal (67%).

The relation of retinal artery occlusion and carotid artery stenosis.

We retrospectively studied 46 patients with symptomatic retinal artery occlusion and assessed the pattern and extent of carotid artery disease ipsilateral to the retinal artery occlusion. Ipsilateral internal carotid artery atherosclerotic lesions were virtually limited to the cervical arterial segment; 50% of such lesions were plaques or stenoses of less than or equal to 60%, whereas 15% of the angiograms were normal. No clinical features were significantly associated with a flow-limiting carotid stenosis of greater than 60%.

Painful tonic spasms caused by putaminal infarction.

Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemichorea-hemiballismus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb.

Intracranial tumors: unexpected uptake of I-123 HIPDM.

I-123 HIPDM is a brain imaging agent thought to reflect cerebral cortical perfusion. Increased uptake of I-123 HIPDM occurred unexpectedly in two cases of intracranial tumors.

Adult intrasellar teratoid tumor.

Intracranial teratomas rarely occur in adults. The most common sites are the pineal followed by the suprasellar or hypothalamic areas. Infrequently, teratomas can arise within the sella turcica and mimic a pituitary adenoma or craniopharyngioma.

Transient Passovoy defect during a febrile illness.

The Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein-Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal.

Isolated facial myokymia and facial contracture: computed tomography and magnetic resonance imaging correlation.

Isolated facial myokymia with contracture can be the earliest manifestation of intrinsic lesions of the brainstem. We report a case of facial myokymia with contracture occurring as the result of a pontine glioma, as depicted on cranial computed tomography and magnetic resonance imaging studies. The rostral location of the tumor supports the supranuclear disinhibition hypothesis of facial myokymia.

Long survival of primary cerebral lymphoma with progressive radiation necrosis.

A 56-year-old man lived 8 years after excision and irradiation of a primary cerebral lymphoma. Delayed radiation necrosis caused progressive neurologic deterioration and probably his steroid-responsive episodes of obtundation. Vasogenic edema induced by radiation may account for the latter.