Hemisensory loss in myasthenia gravis.

A 73-year-old white man presents with left-sided ptosis and diplopia in the absence of ophthalmoplegia, with left hemibody paresthesia. He reports intermittent dysphagia and dizziness for 1 month and diarrhoea for 2 months. Serum and electrodiagnostic studies confirmed the diagnosis of myasthenia gravis. This case highlights the non-classic presentation of myasthenia gravis in the absence of ophthalmoplegia with a unique unexplained hemisensory deficit.

Exploding Head Syndrome as Aura of Migraine with Brainstem Aura: A Case Report.

This article reports a case of exploding head syndrome (EHS) as an aura of migraine with brainstem aura (MBA). A middle-aged man presented with intermittent episodes of a brief sensation of explosion in the head, visual flashing, vertigo, hearing loss, tinnitus, confusion, ataxia, dysarthria, and bilateral visual impairment followed by migraine headache. The condition was diagnosed as MBA. Explosive head sensation, sensory phenomena, and headaches improved over time with nortriptyline. This case shows that EHS can present as a primary aura symptom in patients with MBA.

Permanent Cerebellar Degeneration After Acute Hyperthermia with Non-toxic Lithium Levels: a Case Report and Review of Literature.

This was a study of a 33-year-old man with bipolar disorder treated with lithium who developed cerebellar atrophy after an event of extreme hyperthermia. Unlike previously reported cases of acute cerebellar atrophy after heat stroke, neuroleptic syndrome or lithium toxicity, this case was characterized by a chronic cerebellar atrophy that developed after sepsis-induced hyperthermia in the setting of non-toxic lithium levels. Unique to this case also was the early finding of cerebellar atrophy on MRI 2 weeks after the episode of hyperthermia, long-term neurotoxicity after the novo lithium therapy, and longest follow-up case of chronic cerebellar syndrome after hyperthermia with non-toxic lithium levels.

Chronic inhibitory effect of riluzole on trophic factor production.

Riluzole is the only FDA approved drug for the treatment of amyotrophic lateral sclerosis (ALS). However, the drug affords moderate protection to ALS patients, extending life for a few months by a mechanism that remains controversial. In the presence of riluzole, astrocytes increase the production of factors protective to motor neurons. The stimulation of trophic factor production by motor neuron associated cells may contribute to riluzole's protective effect in ALS. Here, we investigated the effects of media conditioned by astrocytes and Schwann cells acutely or chronically incubated with riluzole on trophic factor-deprived motor neuron survival. While acute riluzole incubation induced CT-1 secretion by astrocytes and Schwann cells, chronic treatment stimulated a significant decrease in trophic factor production compared to untreated cultures. Accordingly, conditioned media from astrocytes and Schwann cells acutely treated with riluzole protected motor neurons from trophic factor deprivation-induced cell death. Motor neuron protection was prevented by incubation with CT-1 neutralizing antibodies. In contrast, conditioned media from astrocytes and Schwann cells chronically treated with riluzole was not protective. Acute and chronic treatment of mice with riluzole showed opposite effects on trophic factor production in spinal cord, sciatic nerve and brain. There was an increase in the production of CT-1 and GDNF in the spinal cord and CT-1 in the sciatic nerve during the first days of treatment with riluzole, but the levels dropped significantly after chronic treatment with the drug. Similar results were observed in brain for CT-1 and BDNF while there was no change in GDNF levels after riluzole treatment. Our results reveal that riluzole regulates long-lasting processes involving protein synthesis, which may be relevant for riluzole therapeutic effects. Changing the regimen of riluzole administration to favor the acute effect of the drug on trophic factor production by discontinuous long-term treatment may improve the outcome of ALS patient therapy.

Corticosteroid treatment of mitochondrial encephalomyopathies.

BACKGROUND: Mitochondrial encephalopathy with lactic acidosis and stroke-like symptoms (MELAS) is a multisystem disorder characterized by stroke-like episodes, seizures, dementia, headaches,evidence of mitochondrial myopathy. Lactic acidosis and ragged red fibers are often present. A variety of therapies have been used with inconclusive and disappointing results. There have been very few cases of MELAS reported as corticosteroid responsive. SUMMARY: A 27-year-old healthy man was hospitalized with recurrent generalized tonic-clonic seizures, intractable headaches, and stroke-like symptoms. These symptoms improved after the initiation of corticosteroid treatment. Multiple attempts to taper corticosteroids were followed by clinical relapse. Genetic testing in this patient revealed a point mutation at nt 3243 of the mitochondrial tRNA gene, confirming the diagnosis of MELAS. The patient died 1 year later after an episode of status epilepticus. Corticosteroid therapy in individual MELAS patients has been associated with clinical improvement. Discrepancy exists with regard to corticosteroid type, dose, route of administration, length of therapy, patient population, and clinical and metabolic parameters to follow during corticosteroid therapy. CONCLUSIONS: Corticosteroid therapy may be beneficial during acute exacerbation of MELAS.