Transcutaneous access to retrograde axonal flow.

Transcutaneous entry of fluorescent tracer and subsequent retrograde neuronal transport achieved by the use of dimethyl sulfoxide (DMSO) as a vehicle fluorescent dye dissolved in DMSO and applied topically to the hind limb of rats was found in corresponding dorsal root ganglia; aqueous absorption of tracer dye by neuronal tissue was not demonstrated. This example of transcutaneous access and retrograde transport may have implications as to the entry of various toxins, viruses, chemicals, and therapeutic agents to the nervous system.

Improvement in the polyneuropathy associated with familial amyloid polyneuropathy after liver transplantation.

OBJECTIVE: To study, following liver transplantation, the neurologic progression or regression of the polyneuropathy in a cohort of patients with familial amyloidotic polyneuropathy (FAP). BACKGROUND: FAP is characterized by the relentless progression of neurologic and cardiac impairment, leading to death within 7 to 15 years after disease onset. No effective treatment to slow or halt the progression of this disease has been found to date. DESIGN/METHODS: Over the past 3 years, our FAP patients were offered liver transplantation as treatment. We report on nine patients who were followed longitudinally with serial neurologic examinations since transplantation. RESULTS: Clinically, all patients evaluated for neurologic progression reported significant improvement in general well being. No patient showed any progression in neurologic disease since receiving a liver transplant. Improvements are documented in symptomatic, autonomic, and sensorimotor neurologic disease in all patients. CONCLUSION: Our experience suggests that liver transplantation may offer hope for arrest of progression and neurologic improvement in patients with FAP.

Alpha Interferon in AIDS-Related Progressive Multifocal Leukoencephalopathy.

OBJECTIVE: To determine the efficacy of recombinant interferon alpha in the treatment of progressive multifocal leukoencephalopathy associated with the acquired immunodeficiency syndrome (AIDS). DESIGN: Open label, uncontrolled study. SETTING: Neurological unit and clinical AIDS program, Boston City Hospital, Boston, MA. PATIENTS: Four consecutive AIDS patients with pathologically confirmed progressive multifocal leukoencephalopathy. INTERVENTION: Each patient received alpha interferon for 4-12 weeks in a dose of 5-10 million units daily, administered subcutaneously. In addition, two of the four were taking acyclovir 2400 mg/day orally over the same period. RESULTS: None of the patients showed any clinical response to the therapy; the mean survival was 14 weeks. No adverse effects of the treatment were encountered. CONCLUSIONS: Despite anecdotal evidence that alpha interferon is effective in the treatment of progressive multifocal leukoencephalo pathy in non-AIDS patients, the experience of these patients suggests that the drug is of no benefit in AIDS-related PML.

Classification of peripheral neuropathy: the long and the short of it.

Insights into how the nervous system functions have often sprung from knowledge of the localization of nervous system diseases. Indeed, the recent rebirth of behavioral neurology has been sparked by new insights into the topography of higher cortical functions. Yet, the localization of disease affecting the peripheral nervous system is simply assumed to be a by-product of the beginning course in gross anatomy! Indeed, the anatomic sophistication laid out by Sunderland and reflected in the daily practice of electromyography might cause doubt as to the need for further inquiry into the problem of localization of disease within peripheral nervous system. There are, however, numerous problems in clinical practice that do not yield to the traditional root-plexus-nerve analysis of localization.

Temporal arteritis-like presentation of carotid atherosclerosis.

A 68 year-old woman presented with a two-week history of amaurosis fugax, ipsilateral fronto-temporal headache and jaw claudication suggesting carotid giant cell arteritis. However, this syndrome proved to be due to atherosclerosis causing complete occlusion of the external carotid artery at its origin and narrowing of the internal carotid artery. Combined external and internal carotid endarterectomy relieved the symptoms. The symptom complex of temporal arteritis may be rarely mimicked by carotid atherosclerotic occlusive disease.

Universal dissociated anesthesia due to bilateral brain-stem infarcts.

A 59-year-old man had loss of pin-prick and temperature sensation over his head, face, neck, trunk, and all extremities while light touch, vibration, joint position sense, and deep pain were preserved. This was the cumulative result of infarctions in the territories of the right superior cerebellar and left posterior inferior cerebellar arteries that occurred three years apart. To our knowledge, a syndrome of bilateral discrete interruption of spinothalamic tracts and of the spinal tracts of the trigeminal system in the brain stem has not been reported. Dissociated sensory loss enveloping the entire body accompanied by truncal and limb ataxia without weakness demonstrated a striking clinical picture. The preservation of deep somatic and visceral pain when cutaneous pain sensation was lost was another notable feature. We review the causes of widespread dissociated sensory loss and discuss the implications of the dissociation of deep from superficial pain.

Lithium in aggressive behavior.

A patient with intractable aggression was treated with lithium carbonate. On the basis of the authors' study and their review of the literature, they conclude that lithium may exacerbate the interictal aggression sometimes noted in patients with temporal lobe spike activity.

Tonic fecal seizures in nonketotic hyperglycemia of diabetes mellitus.

Three patients had recurrent focal motor seizures as the first manifestation of nonketotic hyperglycemia (NKHG) of diabetes mellitus. The seizures were characterized by stereotypical tonic changes in body posture and arrest of speech that have been associated with supplementary motor area seizures. Recognition of the link between this unusual form of focal epilepsy and NKHG would help in the early diagnosis and treatment of the serious underlying metabolic disturbance.

Diabetic truncal polyneuropathy.

Diabetic truncal polyneuropathy is a clinical entity characterized by sensory deficit in the distribution of the thoracic intercostal nerves. The sensory loss is relatively symmetric and involves multiple thoracic dermatomes, beginning close to the anterior midline. Diabetic truncal polyneuropathy occurs in patients with advanced distal polyneuropathy involving the limbs. This entity is important because it can be confused with myelopathies that produce sensory levels over the torso. Moreover, recognition of diabetic truncal polyneuropathy is important since this disorder is associated with autonomic neuropathy.