Camila C. Aquino

Clinical spectrum of levodopa-induced complications.

The first years of Parkinson disease (PD) treatment are marked by good and sustained responses to dopaminergic therapy. With disease progression and longer exposure to levodopa (l‐dopa), patients develop a range of l‐dopa–induced complications that include motor and non‐motor symptoms. Motor complications include motor fluctuations, characterized by periods of reduced benefit from the medication, and l‐dopa–induced dyskinesia, characterized by emergence of hyperkinetic involuntary movements. Dyskinesia can occur at peak effect of l‐dopa, at the beginning and end of dose, or between doses. These motor complications are often associated with fluctuations in non‐motor symptoms, particularly fluctuations in neuropsychiatric, autonomic, and sensory symptoms. Recognizing such complications and understanding their relationship with the timing of l‐dopa doses is essential for adequate diagnosis and management.

Tardive dyskinesia syndromes: current concepts

Tardive syndromes (TS) encompass a broad spectrum of abnormal movements due to chronic exposure to dopamine receptor blocking agents. This review provides a compiled update on TS, including phenomenology, epidemiology, pathophysiology, genetic correlations and therapeutics, highlighting the emerging experience with atypical antipsychotics. The advent of atypical antipsychotics, which have lower affinity for dopamine receptors and act on 5-HT2A and 5-HT2C serotonin receptors, was expected to dramatically reduce the prevalence and incidence of this iatrogenic problem. Recent studies have shown that the reduction has been more modest than expected and TS remains an important challenge. Recent insights on pathophysiology, risk factors and genetic correlations have raised the hope for further individualized treatment for schizophrenic patients, and more strict use of antipsychotics. Up to now, there is no definite treatment for TS, but options range from relatively innocuous low doses of propranolol to more invasive procedures such as deep brain stimulation.

Sleep disorders in cerebellar ataxias

Cerebellar ataxias comprise a wide range of etiologies leading to central nervous system-related motor and non-motor symptoms. Recently, a large body of evidence has demonstrated a high frequency of non-motor manifestations in cerebellar ataxias, specially in autosomal dominant spinocerebellar ataxias (SCA). Among these non-motor dysfunctions, sleep disorders have been recognized, although still under or even misdiagnosed. In this review, we highlight the main sleep disorders related to cerebellar ataxias focusing on REM sleep behavior disorder (RBD), restless legs syndrome (RLS), periodic limb movement in sleep (PLMS), excessive daytime sleepiness (EDS), insomnia and sleep apnea.

Restless Genital Syndrome in Parkinson Disease

IMPORTANCE Symptoms in the genital region, such as pain, discomfort, tingling, and burning sensations, have rarely been reported in Parkinson disease (PD), and the previous cases were attributed to nonmotor off symptoms. We report a patient with PD and severe genital discomfort unrelated to motor fluctuations but compatible with restless genital syndrome. OBSERVATIONS A 65-year-old woman with PD experienced a disabling discomfort in her pelvis and genital region for 3 years. The episodes occurred in the evening and were triggered by sitting or lying down for a period. Gynecological investigation was unrevealing. She experienced improvement with a low dose of a dopamine agonist. CONCLUSION AND RELEVANCE Restless genital syndrome is a rare disorder that can be a source of distress and disability. In patients with PD, restless genital syndrome should be included in the differential diagnosis of genital symptoms and restlessness, along with nonmotor wearing off and akathisia. A detailed clinical history is essential for this diagnosis and treatment with dopamine agonists can provide benefit.

Progressive supranuclear palsy: new concepts

Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.

Ginkgo biloba and cerebral bleeding: a case report and critical review.

Ginkgo biloba is a herbal medication that is often used worldwide. Although side effects are uncommon, G. biloba has been associated with serious bleeding complications, especially intracranial hemorrhage. We report the case of a young woman who made chronic use of G. biloba and suffered from cerebral bleeding without any structural abnormalities. Several studies have pointed to the association between G. biloba and intracranial hemorrhage.

Prevalence and impact of headache and migraine among Pomeranians in Espirito Santo, Brazil

This is the first study to assess the prevalence of headache and migraine among Pomeranian descendents in Brazil. A high prevalence of headache in the last 6 months was found (53.2%). Most headache sufferers were diagnosed as having migraine (55%). More women reported to have headache than men (65% and 33.8%, respectively). Migraine was the most common headache found among women (62.2%). Among men migraine was responsible for only 37.8% of the cases of headache. A high impact of headache was found, especially among migraineurs. Most of the headache sufferers declared to seek medical assistance for headache (67%) and most of them used to take common analgesics for headache relief. None of them was under prophylactic therapy.

Hepatitis C virus: a rare manifestation--remitting relapsing central and peripheral demyelination.

The most frequent neurologic manifestations of hepatitis C virus infection include peripheral neuropathy axonal type and central nervous system (CNS) vasculitis. Affected patients usually have cryoglobulinemia and other signs of vasculitis. Demyelinating lesions, both central and peripheral are rarely described. We present a case of simultaneous peripheral nervous system and CNS demyelination that comes in relapsing episodes, with negative cryoglobulins.

A double-blind randomized controlled trial of low doses of propranolol, nortriptyline, and the combination of propranolol and nortriptyline for the preventive treatment of migraine

Few trials have evaluated combination of two or more drugs in the preventive treatment of migraine. In this study three therapeutic regimens were compared: (a) propranolol, at a dose of 40 mg per day, (b) nortriptyline, at a dose of 20 mg per day, and (c) the combination of these two drugs in these dosages. The groups were matched according to age, gender, and frequency of migraine attacks prior to treatment. The period of treatment was two months and the frequency and intensity of headache attacks of the 30 days pre-treatment period were compared with the frequency of headaches in the treatment period. Fourteen patients in groups A and B and sixteen patients in group C have completed the study. Treatment with propranolol, alone or in combination, was shown to be effective. Treatment with nortriptyline alone was not effective. All three therapeutic regimens were safe and side effects were minimal. The frequency of discontinuation of the study was the same in the 3 groups but no patient left the study due to adverse reactions. The combined therapy proved to be as safe as the monotherapy. Further studies evaluating this and other possible combinations of drugs in higher doses and for longer periods, should more clearly elucidate the role of combined therapy in the treatment of migraine.

Deep Brain Stimulation in Rare Inherited Dystonias

BACKGROUND Rare causes of inherited movement disorders often present with a debilitating phenotype of dystonia, sometimes combined with parkinsonism and other neurological signs. Since these disorders are often resistant to medications, DBS may be considered as a possible treatment. METHODS Patients with identified genetic diseases (ataxia-telangiectasia, chorea-achantocytosis, dopa-responsive dystonia, congenital nemaline myopathy, methylmalonic aciduria, neuronal ceroid lipofuscinosis, spinocerebellar ataxia types 2 and 3, Wilsons disease, Woodhouse-Sakati syndrome, methylmalonic aciduria, and X trisomy) and disabling dystonia underwent bilateral GPi DBS (bilateral thalamic Vim nucleus in 1 case). The primary outcome was the difference in the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) between baseline, 1 year and last available follow-up. Preoperative factors such as age at surgery, disease duration at surgery, proportion of life lived with dystonia and severity of dystonia were correlated to the primary outcome. RESULTS Eleven patients were operated between February 2003 and December 2013. Age and duration of disease at time of surgery were 30 ± 19 and 12.5 ± 15.7 years, respectively. DBS effects on dystonia severity were variable but overall marginally effective, with a mean improvement of 7.9% (p = 0.39) at 1-year follow-up and 16.7% (p = 0.46) at last follow-up (mean 47.3 ± 19.9 months after surgery). No preoperative factors were identified to predict the surgical outcome. CONCLUSION Our findings support the current knowledge that DBS is modestly effective in treating rare inherited dystonias with a combined phenotype. However, the BFMDRS might not be the best tool to measure outcome in these severely affected patients.