Case reports in neurology
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Case reports in neurology · May 2014
Case ReportsRecurrence of Susac Syndrome following 23 Years of Remission.
Susac syndrome is an autoimmune microangiopathy affecting the brain, retina and inner ear (cochlea and semicircular canals), leading to encephalopathy, branch retinal artery occlusions (BRAOs) and asymmetric neurosensory hearing loss, respectively. The natural history and long-term prognosis are variable as the disease has been shown to be monophasic and self-limiting, polycyclic or chronic continuous. We describe a 35-year-old woman who presented with a sudden hearing loss in the left ear in the 37th week of her second pregnancy. ⋯ We expand on the variability in the course of Susac syndrome as recurrence may occur after as long as 23 years. Cases of monophasic self-limiting Susac syndrome may in fact turn polycyclic with an interval of more than 2 decades between the bouts of the disease. In these cases, suspecting the development of exacerbation early is important in order to start the treatment promptly.
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Case reports in neurology · Jan 2014
Vocalization in dementia: a case report and review of the literature.
Vocalizations are part of the spectrum of the 'negative' behavioral and psychological symptoms of dementia (BPSD). We describe a patient with moderate-stage mixed dementia of Alzheimer's disease and cerebrovascular disease and a left orbitofrontal lesion exhibiting vocalization. The use of 'redirection' has been demonstrated to be an effective nonpharmacological means of controlling BPSD, while reducing caregiver distress. ⋯ In patients with dementia exhibiting negative symptoms of BPSD, using nonpharmacologic techniques (i.e. redirection) may be indicated. Psychotropic medications rarely address negative BPSD symptoms, while simultaneously decreasing patient's quality of life. Nonpharmacologic approaches are beneficial as first-line therapy for negative BPSD.
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Case reports in neurology · Jan 2013
Case ReportsDynamic J-Point Elevation Associated with Epileptic Hemiplegia: The Osborn Wave of Todd's Paralysis.
THIS CASE REPORT EXPOSES A PHENOMENON WHICH, ALTHOUGH PROPOSED, HAS NOT BEEN DESCRIBED IN CLINICAL LITERATURE: transient postictal hemiplegia (Todd's paralysis) with concomitant electrocardiographic J-point deflection (Osborn waves). Although typically associated with hypothermia, a prominent J-wave on the electrocardiogram (ECG) results from a transmyocardial voltage gradient during ventricular repolarization. ⋯ The same central nervous system autonomic dysfunction has been theorized to produce subendocardial hypoperfusion with electrocardiographic change and cardiac troponin T elevation. This is the first described ECG evidence of a dynamically displaced J-point in the setting of postictal hemiplegia.
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Myasthenia gravis (MG) is an autoimmune disorder characterized by weakness in specific muscle groups, especially the ocular and bulbar muscles. Guillain-Barré syndrome (GBS) presents with ascending paralysis and areflexia, often secondary to an infection. Several theories have been proposed regarding the etiology behind GBS, with many studies pointing to a possible autoimmune cause. ⋯ Although the diagnosis of MG was confirmed by the positive anticholinesterase antibodies and tensilon test, several features, including sudden onset of ascending paralysis and areflexia, were more common in GBS than MG. It is possible, albeit rare, that these two syndromes could have developed concurrently and that the untreated diabetes mellitus could have contributed to the neurological symptoms. This case is reported because of the rarity of its features, diagnostic and management challenges.
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Case reports in neurology · Sep 2012
Case ReportsFatal hyperammonemic brain injury from valproic Acid exposure.
Hyperammonemia is known to cause neuronal injury, and can result from valproic acid exposure. Prompt reduction of elevated ammonia levels may prevent permanent neurological injury. We report a case of fatal hyperammonemic brain injury in a woman exposed to valproic acid. ⋯ Cerebral edema secondary to hyperammonemia is potentially reversible if recognized early. Ammonia excretion can be facilitated by initiation of hemodialysis and administration of scavenging agents (sodium phenylacetate and sodium benzoate). Severe hyperammonemia can result from valproic acid exposure even in the absence of hepatotoxicity or inborn errors of metabolism. It is important to check serum ammonia in any patient with encephalopathy who has had recent valproic acid exposure.