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Cannabis has been used for many medicinal purposes, including management of spasms, dystonia, and dyskinesias, with variable success. Its use for tetanus was described in the second century BCE, but the literature continues to include more case reports and surveys of its beneficial effects in managing symptoms of hyperkinetic movement disorders than randomized controlled trials, making evidence-based recommendations difficult. This paper reviews clinical research using various formulations of cannabis (botanical products, oral preparations containing ∆(9)-tetrahydrocannabinol and/or cannabidiol) and currently available preparations in the USA (nabilone and dronabinol). This has been expanded from a recent systematic review of cannabis use in several neurologic conditions to include case reports and case series and results of anonymous surveys of patients using cannabis outside of medical settings, with the original evidence classifications marked for those papers that followed research protocols. Despite overlap in some patients, dyskinesias will be treated separately from dystonia and chorea; benefit was not established beyond individual patients for these conditions. Tics, usually due to Tourettes, did respond to cannabis preparations. Side effects reported in the trials will be reviewed but those due to recreational use, including the dystonia that can be secondary to synthetic marijuana preparations, are outside the scope of this paper.

A patient's hormonal milieu contributes to the timing of emergence of several epilepsy syndromes that are known to begin at puberty and recede with the end of reproductive potential. One's hormonal balance at any particular moment contributes to seizure occurrence in both men and women. The best studied condition, catamenial epilepsy, refers to seizure clusters occurring in a cyclical pattern related to menses. Treatment of epilepsy using hormones complements standard antiepileptic therapy and its use will be reviewed, along with some other medications unique to catamenial epilepsy, such as diuretics.Seizures and “silent” epileptiform discharges in turn affect the hypothalamic pituitary axis and can cause release of hormones at inappropriate times leading to sexual dysfunction, menstrual irregularity, infertility and premature termination of reproductive states. Combined with psychological consequences of epilepsy, this sexual dysfunction has deleterious effects on the quality of life in patients and their partners.

Objective: To determine the efficacy of medical marijuana in several neurologic conditions., Methods: We performed a systematic review of medical marijuana (1948-November 2013) to address treatment of symptoms of multiple sclerosis (MS), epilepsy, and movement disorders. We graded the studies according to the American Academy of Neurology classification scheme for therapeutic articles., Results: Thirty-four studies met inclusion criteria; 8 were rated as Class I., Conclusions: The following were studied in patients with MS: (1) Spasticity: oral cannabis extract (OCE) is effective, and nabiximols and tetrahydrocannabinol (THC) are probably effective, for reducing patient-centered measures; it is possible both OCE and THC are effective for reducing both patient-centered and objective measures at 1 year. (2) Central pain or painful spasms (including spasticity-related pain, excluding neuropathic pain): OCE is effective; THC and nabiximols are probably effective. (3) Urinary dysfunction: nabiximols is probably effective for reducing bladder voids/day; THC and OCE are probably ineffective for reducing bladder complaints. (4) Tremor: THC and OCE are probably ineffective; nabiximols is possibly ineffective. (5) Other neurologic conditions: OCE is probably ineffective for treating levodopa-induced dyskinesias in patients with Parkinson disease. Oral cannabinoids are of unknown efficacy in non-chorea-related symptoms of Huntington disease, Tourette syndrome, cervical dystonia, and epilepsy. The risks and benefits of medical marijuana should be weighed carefully. Risk of serious adverse psychopathologic effects was nearly 1%. Comparative effectiveness of medical marijuana vs other therapies is unknown for these indications.

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

In principle, the use of anticonvulsant drugs does not differ between acute and remote symptomatic seizures, but control of acute symptomatic seizures requires simultaneous treatment of the underlying etiology. Prevention of remote seizures when the risk is known to be high has been the subject of intense efforts at antiepileptogenesis, but the optimal duration of treatment after an injury is not yet known. Appropriate evaluation of a seizure depends on individual circumstances, but findings on examination, laboratory tests (serum electrolytes, magnesium, glucose, assessment of hepatic and renal function), and brain imaging (CT scan or MRI) are necessary to determine the most likely cause. Lumbar puncture is always required when there is suspicion of meningitis or encephalitis. Preferred medications for treatment of acute symptomatic seizures or status epilepticus are those available for intravenous use, such as benzodiazepines, fosphenytoin or phenytoin, valproate, levetiracetam, and phenobarbital. Diazepam is also available as a gel for rectal administration. Seizures that occur in patients with epilepsy because of missed antiepileptic drugs or inadequate serum levels should be treated with additional doses of their regular medications; loading doses can be administered with minimal toxicity in tolerant patients. Surgery is rarely necessary in the acute setting except for intracerebral lesions with rapidly rising intracranial pressure and impending herniation. After seizures are controlled, the provoking condition must also be determined and treated.

A committee assembled by the American Academy of Neurology (AAN) reassessed the evidence related to the care of women with epilepsy (WWE) during pregnancy, including antiepileptic drug (AED) teratogenicity and adverse perinatal outcomes. It is highly probable that intrauterine first-trimester valproate (VPA) exposure has higher risk of major congenital malformations (MCMs) compared to carbamazepine (CBZ), and possibly compared to phenytoin (PHT) or lamotrigine (LTG). It is probable that VPA as part of polytherapy and possible that VPA as monotherapy contribute to the development of MCMs. AED polytherapy probably contributes to the development of MCMs and reduced cognitive outcomes compared to monotherapy. Intrauterine exposure to VPA monotherapy probably reduces cognitive outcomes and monotherapy exposure to PHT or phenobarbital (PB) possibly reduces cognitive outcomes. Neonates of WWE taking AEDs probably have an increased risk of being small for gestational age and possibly have an increased risk of a 1-minute Apgar score of <7. If possible, avoidance of VPA and AED polytherapy during the first trimester of pregnancy should be considered to decrease the risk of MCMs. If possible, avoidance of VPA and AED polytherapy throughout pregnancy should be considered and avoidance of PHT and PB throughout pregnancy may be considered to prevent reduced cognitive outcomes.

A committee assembled by the American Academy of Neurology (AAN) reassessed the evidence related to the care of women with epilepsy (WWE) during pregnancy, including the risk of pregnancy complications or other medical problems during pregnancy, change in seizure frequency, the risk of status epilepticus, and the rate of remaining seizure-free during pregnancy. The committee evaluated the available evidence according to a structured literature review and classification of relevant articles. For WWE who are taking antiepileptic drugs (AEDs), there is probably no substantially increased risk (>2 times expected) of cesarean delivery or late pregnancy bleeding, and probably no moderately increased risk (>1.5 times expected) of premature contractions or premature labor and delivery. There is possibly a substantially increased risk of premature contractions and premature labor and delivery during pregnancy for WWE who smoke. WWE should be counseled that seizure freedom for at least 9 months prior to pregnancy is probably associated with a high likelihood (84-92%) of remaining seizure-free during pregnancy. WWE who smoke should be counseled that they possibly have a substantially increased risk of premature contractions and premature labor and delivery.

A committee assembled by the American Academy of Neurology (AAN) reassessed the evidence related to the care of women with epilepsy (WWE) during pregnancy, including preconceptional folic acid and prenatal vitamin K use and the clinical implications of placental and breast-milk transfer of antiepileptic drugs (AEDs). The committee evaluated the available evidence based on a structured literature review and classification of relevant articles. Preconceptional folic acid supplementation is possibly effective in preventing major congenital malformations in the newborns of WWE taking AEDs. There is inadequate evidence to determine if the newborns of WWE taking AEDs have a substantially increased risk of hemorrhagic complications. Primidone and levetiracetam probably transfer into breast milk in clinically important amounts. Valproate, phenobarbital, phenytoin, and carbamazepine probably are not transferred into breast milk in clinically important amounts. Pregnancy probably causes an increase in the clearance and a decrease in the concentrations of lamotrigine, phenytoin, and, to a lesser extent carbamazepine, and possibly decreases the level of levetiracetam and the active oxcarbazepine metabolite, the monohydroxy derivative (MHD). Supplementing WWE with at least 0.4 mg of folic acid before pregnancy may be considered. Monitoring of lamotrigine, carbamazepine, and phenytoin levels during pregnancy should be considered, and monitoring of levetiracetam and oxcarbazepine (as MHD) levels may be considered. A paucity of evidence limited the strength of many recommendations.

Purpose: Previous reports have suggested that hormone replacement therapy (HRT) could increase seizure activity in women with epilepsy. We sought to determine whether adding HRT to the medication regimen of postmenopausal women with epilepsy was associated with an increase in seizure frequency., Methods: This was a randomized, double-blind, placebo-controlled trial of the effect of HRT on seizure frequency in postmenopausal women with epilepsy, taking stable doses of antiepileptic drugs (AEDs), and within 10 years of their last menses. After a 3-month prospective baseline, subjects were randomized to placebo, Prempro (0.625 mg of conjugated equine estrogens plus 2.5 mg of medroxyprogesterone acetate or CEE/MPA) daily, or double-dose CEE/MPA daily for a 3-month treatment period., Results: Twenty-one subjects were randomized after completing baseline. The subjects' ages ranged from 45 to 62 years (mean, 53 years; SD, +/-5), and the number of AEDs used ranged from none to three (median, one). Five (71%) of seven subjects taking double-dose CEE/MPA had a worsening seizure frequency of at least one seizure type, compared with four (50%) of eight taking single-dose CEE/MPA and one (17%) of six taking placebo (p = 0.05). An increase in seizure frequency of the subject's most severe seizure type was associated with increasing CEE/MPA dose (p = 0.008). An increase in complex partial seizure frequency also was associated with increasing CEE/MPA dose (p = 0.05). Two subjects taking lamotrigine had a decrease in lamotrigine levels of 25-30% while taking CEE/MPA., Conclusions: CEE/MPA is associated with a dose-related increase in seizure frequency in postmenopausal women with epilepsy. CEE/MPA may decrease lamotrigine levels.

Objective: To clarify the relationship between fractures and antiepileptic drug (AED) use., Methods: Menopausal women with epilepsy were interviewed at two clinics regarding site, year and circumstances of any fracture, duration of AED use and menopause. Fracture sites were analyzed according to AED use., Results: Twenty-nine fractures occurred in 20 of the 50 interviewed subjects (mean age 54). Nine occurred prior to AEDs; seven attributed to accident and two to clumsiness. Twenty occurred on AEDs; 10 attributed to clumsiness (most in the leg and foot), eight to seizure (most in the arm or hand) and two to accident. Duration of AED exposure was similar in both groups and in osteoporotic vs non-osteoporotic sites., Conclusions: Epilepsy therapy may contribute more to the lifetime occurrence of fracture than seizures themselves. More screening for osteoporosis is required. While adjusting doses to prevent seizures, ongoing screening for neurotoxicity must be maintained in order to avoid fractures.

Purpose: To determine the risk of seizures in critically ill patients receiving the antibiotic imipenem, a broad-spectrum antibiotic that has been associated with seizures. Reports generally have not considered other contributing factors such as dose, seizure history, and morbidity index of the underlying illness necessitating the antibiotic., Methods: Charts of all patients in a 450-bed municipal hospital who received imipenem in a 6-month period, as determined by pharmacy records, were reviewed for dosage and duration of imipenem use, occurrence of seizures. and mortality outcome. Attention was paid to demographic features; pattern of seizure occurrence during, before, and after imipenem use; renal function; and correction for dosage based on size., Results: Seventy-five charts were reviewed. Sixty-three patients had no seizures during the hospitalization, four had seizures while receiving imipenem, and eight had seizures during the hospitalization but before or after imipenem use. The incidence of seizures was 4/1,000 patient-days on, and 3.9/1,000 patient-days off imipenem (not significant). The risk of seizure in both groups was considerably higher in those patients with a history of seizures before hospitalization. The presence of other factors that could contribute to increased concentration of imipenem in the brain, such as renal failure or acute stroke, did not contribute to seizure incidence. Metabolic derangement, anoxia, and phenytoin discontinuation did contribute to seizure incidence., Conclusions: Seizure incidence is increased in all critically ill patients (16% of patients studied), but with no added risk during the period patients received imipenem. Determining the proper dose based on a patient's body mass, correction of dose in the presence of renal failure, and avoidance of excess of 2 g/day of imipenem removes any added risk for seizures from imipenem. Despite experimental data to suggest action of imipenem on the glutamate/N-methyl-d-aspartate receptor, or interference with binding to the gamma-aminobutyric acid receptor, and early clinical studies that warned against its use because of seizure risk, we found that careful use of this antibiotic is safe.

A case of a 64-year-old man is presented with painless dysphagia and loud noise on swallowing due to large anterior cervical osteophytes demonstrated on plain radiographs and magnetic resonance imaging accompanied by a brief review of the literature.

A case is reported of a young man who developed bilateral symmetrical basal ganglia infarcts after intravenous use of cocaine and heroin. Ischemic infarcts of the brain are a known complication of to cocaine use, alone or in combination with heroin (speed balling). This symmetrical occurrence of infarction, however, is unusual and has not been reported after cocaine use.

Purpose: Cocaine can provoke seizures, exacerbate a preexisting seizure disorder, or cause an ischemic or hemorrhagic stroke that leads to seizures. To determine the importance of cocaine use in patients with and without epilepsy, we studied these relations and other risk factors for seizures and the mode of cocaine use., Methods: We reviewed all charts of emergency department visits and hospitalizations of patients with discharge diagnoses simultaneously listing seizures, epilepsy, and cocaine use during a 24-month period. Data collected included patient age, sex, route of cocaine use, seizure description and duration of epilepsy, provocative factors, results of electroencephalography and computed tomography, treatment, and outcome., Results: Of 67,668 adult emergency department visits and 25,768 adult admissions, 1,900 were cocaine related, and 58 of these also had seizures or epilepsy. Seizure occurrences were approximately equally distributed among groups with idiopathic epilepsy, remote symptomatic localization-related epilepsy, cerebrovascular disease, and acute symptomatic seizures due to cocaine use alone. Less frequently, seizures were cryptogenic or symptomatic of metabolic abnormalities., Conclusions: Cocaine use can reduce seizure threshold in patients with underlying epilepsy as a direct toxic effect or indirectly by contributing to poor compliance with antiepileptic drug treatment, poor diet, or poor sleep habits. In 12 of the 58 patients, cocaine appeared to be the only provocative factor. This may be a less significant risk factor for epilepsy than either alcohol or head trauma.

Objectives: To describe a patient with a spontaneous spinal epidural hematoma associated with cocaine use and to present issues related to pathogenesis and management of this potential complication., Design: Case report., Setting: Municipal hospital., Case: A spontaneous spinal epidural hematoma developed at the thoracic level in a 62-year-old man in association with cocaine use. Clinical, laboratory, and radiologic data are presented., Main Outcome and Results: The patient was treated medically with intravenous and oral dexamethasone sodium phosphate. His neurologic status gradually improved during a 12-day hospitalization., Conclusion: Cocaine use should be considered in the evaluation of spontaneous spinal epidural hemorrhage.

Subacute sclerosing panencephalitis (SSPE) had largely disappeared from the United States because of nearly universal measles vaccination, but it has reemerged in children infected with human immunodeficiency virus (HIV). Two children with SSPE are described. The first was HIV positive and presented with seizures and encephalopathy at the age of 21 months. The second developed myoclonus and dementia at age 4 years; she was not infected with HIV, but her mother had acquired immunodeficiency syndrome. Magnetic resonance imaging findings were nonspecific and could have been compatible with HIV encephalopathy. Electroencephalography was characteristic of SSPE, showing high-voltage, periodic slow-wave complexes and background slowing. The diagnosis of SSPE was confirmed by brain biopsy or high measles antibody titers in the cerebrospinal fluid.

Rhabomyolysis with myoglobinuria has been added relatively recently to the neurologic complications associated with the increased use of cocaine and the introduction of its alkaloid form (crack). This retrospective study reports our experience with 14 patients who presented with rhabdomyolysis after cocaine use in a municipal hospital over a 3-year period. Seven patients used "crack", 2 intravenous and 3 nasal insufflation. All patients but one had hyperthermia, 11 altered mental status, 8 tachycardia, and 4 muscle rigidity. Nine developed renal failure; 3 of these patients died. Two other patients died of cardiorespiratory arrest. Cocaine-related rhabdomyolysis has a high mortality. The observed association with hyperthermia and other central neurologic features resembles the neuroleptic malignant syndrome. Since chronic cocaine use may alter the availability of dopamine either through transmitter depletion or decrease in the number of dopamine receptors, a common pathogenetic mechanism is possible. However, other mechanisms, which are not mutually exclusive but rather frequently overlapping, may play an important role. These include agitation, hyperthermia, adrenergic overstimulation leading to vasoconstriction and ischemia or calcium release from the sarcoplasmic reticulum resulting in increased entry into the muscle cell leading to cell death; in addition, cocaine has direct toxic effect on the muscles.

Use of cocaine in the USA, has reached epidemic proportions since 1983, when "crack" was introduced, its higher potency compared with cocaine HCl has been associated with a tremendous increase in the incidence of strokes. This study reports our experience with 55 cases of neurovascular events (25 ischemic and 30 hemorrhagic) related to cocaine use in 54 patients. Only 15 patients had other risk factors for stroke. Twenty six patients smoked "crack", 10 snorted cocaine and 12 injected it intravenously. Strokes occurred within 3 h of cocaine use in 15 patients with infarcts and 17 with hemorrhages. Ten infarcts occurred after an overnight binge. Of the hemorrhage group 9 were subarachnoid, 16 intracerebral (8 basal ganglia, 7 hemispheric and one brain stem) and 5 intraventricular. Computerized tomography (CT) showed an aneurysm of the anterior communicating artery, as well as one of the vein of Galen. Four aneurysms and 3 AVMs were identified on angiography. CT revealed 15 infarcts; it was normal in 7 patients with pure motor hemiparesis and in 3 with findings consistent with anterior spinal artery infarction. Several mechanisms may be responsible for the cerebrovascular complications. A sudden rise in systemic arterial pressure may cause hemorrhages, frequently in association with an underlying aneurysm or AVM. Vasospasm, arteritis, myocardial infarction with cardiac arrhythmias and increased platelet aggregation may provoke infarcts.

We describe seven patients with cocaine-induced movements, including choreoathetosis, akathisia, and parkinsonism with tremor. All were seen in 2 years at a municipal hospital, during which 701 visits were attributed to complications of cocaine. Dopaminergic changes are hypothesized to cause euphoria, addiction, and abnormal movements.

Brainstem involvement by Toxoplasma gondii is probably more common than other opportunistic infections, although it has not received adequate attention. Identification of toxoplasmosis is essential, as this condition responds to treatment with a significant increase in life expectancy. During the past 4 years, 366 AIDS patients with central nervous system toxoplasmosis diagnosed by biopsy or improvement following empirical treatment were evaluated. Among them, 8 (7 men, 1 woman; aged 25-55 yr) presented with signs of brainstem dysfunction. Six patients presented with an oculomotor nerve palsy and contralateral hemiplegia; 1 had an additional ipsilateral rubral tremor. The seventh patient had complete external ophthalmoplegia and the eighth had Parinaud's syndrome. Computed tomography revealed enhancing lesions in the brainstem of all patients. Seven responded to treatment with pyrimethamine and sulfadiazine.

We have observed an unusual low amplitude, slow and featureless electroencephalogram (EEG) pattern in some human immunodeficiency virus (HIV) infected patients without focal lesions on computerized tomography (CT scan) of the head. Out of 17 cases, 13 with AIDS and 4 with HIV positive status, 6 had low amplitude EEGs with slowing, all in the AIDS group. Nine of the 13 AIDS patients were demented, and 4 of these demented patients had slow verbal responses and mutism, indicating advanced HIV-related dementia. All 4 had low amplitude, slow EEGs. The patients with low amplitude, slow EEGs also had atrophy on CT scan by visual assessment and by measurement of ventricular indices. Of 17 age-matched controls referred for non-specific complaints such as headache and dizziness or for psychiatric disorders, 3 had EEGs read as low amplitude with slowing; two had normal mental status and one was psychotic. Although this EEG pattern is not etiologically specific, it may correlate with advanced dementia and atrophy on CT scan in AIDS patients.

A diabetic patient became hypoglycemic with right hemiparesis during a contrast-enhanced CT scan. The scan showed a nonenhancing hypodensity in the left internal capsule, that disappeared the following day after treatment with glucose. This is consistent with experimental models that show selective vulnerability of various brain regions to hypoglycemia.

Segmental myoclonus arising in the spinal cord occurs with several viral infections, including herpes zoster radiculitis. Usually, abnormal movements follow the rash and require drug treatment to suppress. We report a patient with AIDS in whom arm and shoulder myoclonus preceded herpes zoster involving the same segments contralaterally on two occasions. Myoclonus remitted promptly with antiviral treatment. Unlike in other immunosuppressed patients, encephalitis did not occur after the second episode.

Pseudotumor cerebri, or increased intracranial pressure without a mass lesion, has been associated with hormonal activity but the exact causative relation is still obscure. We report a case of a 15-year-old girl who developed pseudotumor cerebri manifested by headache, visual symptoms and extraocular muscle palsies 3 weeks after recovering from eclampsia. Possible associations with eclampsia and postpartum changes in estrogen, progesterone and prolactin are discussed.

We describe a patient with "rubral" tremor due to a Toxoplasma abscess of the midbrain. Despite treatment that was effective in promoting partial computed tomography resolution of the lesion, the tremor remained unchanged.

Viral myelitis and bacterial epidural infections are common in intravenous drug abusers, but primary infections of the spinal cord are extremely rare. We report a 50-year-old active intravenous drug user who developed tetraplegia from an intramedullary abscess caused by Pseudomonas cepacia. Despite neurosurgical drainage and appropriate antibiotic therapy, no improvement was seen. Earlier intervention and a high index of suspicion is required in patients with a history of intravenous drug abuse and spinal cord symptoms.

Optimal radiologic investigation greatly improves surgical outcome in cases of epidermoid cyst. As the lesion is benign but will recur if total excision is not accomplished, the surgeon must be prepared to resect the entire capsule and not simply drain the cyst. The contribution of neuroimaging and other diagnostic procedures to the preoperative evaluation of a man with posterior fossa cyst is reported.

We report three patients with recurrent tonic-clonic seizures associated with unilateral pulmonary edema. Each lung was involved on separate occasions in one patient. The mechanisms of seizure-related pulmonary edema are discussed. Although accurate diagnosis of unilateral postictal pulmonary edema may be difficult because of its rarity, not all pulmonary abnormalities that follow seizures represent pneumonia.

We describe two patients, one with clumsy hand-dysarthria syndrome and one with ataxic hemiparesis. Both had pontine lesions on CT. Similar clinical syndromes may be seen with lesions of the internal capsule, and it is not possible to predict the lesion's location from clinical information alone. The clinical picture of dysarthria-clumsy hand syndrome or ataxic hemiparesis is also not specific for lacunar infarction.

Central nervous system involvement occurred in 28 of 121 patients with acquired immune deficiency syndrome (AIDS). The major risk factor in this AIDS population was intravenous drug abuse (64%). A neurologic symptom or disability was the principal reason for hospitalization in 16 cases (57%). Three patients had primary lymphoma of the brain and the remainder had opportunistic infections. Patients with focal neurological features usually had toxoplasmosis. Progressive headache and meningeal signs occurred with cryptococcosis. A progressive subacute dementia was probably due to cytomegalovirus. Other infections included atypical mycobacteria, candida, herpes zoster and possible progressive multifocal leukoencephalopathy.

The incidence of spinal epidural abscess and disk space infection appears to be rising in intravenous drug-using patients. We report 18 cases seen over three years in two municipal hospitals. Staphylococcus aureus was the most common infective agent, but two patients had Mycobacterium tuberculosis infection. Computed tomography of the spine facilitates diagnosis. Early treatment improves outcome but host factors play a role in recovery.