Frontotemporal disorders: the expansive panoply of syndromes and spectrum of etiologies.

Background: Frontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse etiologies, pose major challenges in diagnosis and treatments. Recent studies have yielded insights that the etiology in the majority are due to environmental and sporadic causes, rather than genetic in origin.

Superlative Artistic Abilities in a Patient With Post-traumatic Brain Injury.

This case describes a patient who exhibits newfound superlative abilities in painting, music, philosophy, culinary, and performing arts after a traumatic brain injury (TBI) involving the frontal and temporal lobes. Such a dramatic change in de novo artistic behavior after brain injury is rare but has been reported in other patients with frontotemporal dementia, as well as other neurological diseases. Previous studies have shown that mild frontal cortical dysfunction likely plays a role in facilitating creative endeavors and that artistic circuitry is distributed throughout the brain.

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.

Genetic rhabdomyolysis within the spectrum of the Spinocerebellar Ataxia type 2 responsive to pregabalin.

Background: Spinocerebellar Ataxia type 2 is a slowly progressive adult onset ataxia with a broad clinical presentation.

Case Presentation: We describe a man with Spinocerebellar Ataxia type 2 with chronic, severe, and recurrent rhabdomyolysis, as part of the cerebellar ataxia genetic spectrum. Initially rhabdomyolysis was refractory to multiple medications, but entirely resolved and remained in chronic remission with pregabalin.

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.

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.

Intravitreal Injection-Induced Migraine Headaches.

A case of migraine headache triggered by intravitreal injection, and aborted by retrobulbar injection, is reported. To date, migraine and related cephalgia have not been reported after intravitreal injection. Ophthalmologists and neurologists should be aware of this potential sequela of a very common procedure.

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.

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.

Cerebral blood flow changes in depressed patients after treatment with repetitive transcranial magnetic stimulation: evidence of individual variability.

Objective: To elucidate the neural mechanisms of depression.

Background: Despite extensive study, the neurophysiology of the brain's state(s) corresponding to depression remains uncertain.

Methods: HMPAO single photon emission computed tomographic (SPECT) scans were obtained from eight adults diagnosed with major depression resistant to medication (average age 51 years; 4 men) before and immediately after 10 days of 20 Hz repetitive transcranial magnetic stimulation (rTMS) (2000 stimuli/daily 30' treatment).

Immobilization dystonia.

The mechanisms underlying dystonia after injury are unclear. Pain has been implicated as an important factor. We report four patients who exhibited segmental dystonia following removal of a cast, only two of whom experienced pain during casting.