Periodic dystonia

20 authors' personal observations of periodic dystonia, a rare neurological syndrome, are presented. Main clinical manifestations are discussed in detail: the age of the disease debut, provoking factors, duration and frequency of the fits, the motor pattern, daily distribution of paroxysms. The comparison with analogous literary data is given too.     

Offset-induced audiogenic seizures

A relatively stereotyped seizure reaction can be triggered by the "offset" of an intense bell sound in C57BL/6J mice. Susceptibility to this offset-induced audiogenic seizure was found to depend upon the age of the animals tested (higher in older mice) and the duration of the noise exposure (more effective with longer exposure).     

Nonepileptic posttraumatic seizures

BACKGROUND: The aim of this experimental study was to evaluate the risk of tumor recurrence after laparoscopic cecal resection (LCR) of colonic carcinoma in the rat. METHODS: The experimental cancer consisted of one million cells (DHK/K12), incorporated in an extracellular matrix, placed and secured to the cecal serosa in 110 BD9 rats. Four weeks later, all animals were reoperated through a laparotomy to control tumor growth, and animals with diffuse carcinomatosis were excluded. Eligible animals were randomized either to laparoscopic cecal resection (group LCR, n = 10), to open resection (group OCR, n = 13), or to a control group without resection (group C, n = 13). Resection was always considered as macrocopically complete. All animals were killed 4 weeks after the resection to determine the tumor recurrence and quantify carcinomatosis. RESULTS: We noted diffuse carcinomatosis in 70% of rats in groups C and LCR versus 23% in group OCR (p = 0.038). For tumors noted as S- (not extending outside the serosa), diffuse carcinomatosis was observed in all animals of group C (3 of 3), in 6 of 8 in group LCR, and 0 of 6 in group OCR (p = 0.004). The rate of port site or incisional metastases was not significantly different between groups. CONCLUSIONS: These preliminary results demonstrated the deleterious impact of the laparoscopy for resection of large bowel malignancy. LCR increased significantly the incidence of a diffuse carcinomatosis even when performed for locally noninvasive tumors (S-).     

Periodic downbeat nystagmus

The authors observed a periodic downbeat nystagmus with a cycle of 3 minutes 30 seconds, beating downward for a period of 90 seconds every 2 minutes. It lasted 86 hours in a patient with severe hypomagnesemia associated with complications from scleroderma. There was no lesion on brain imaging, and an EEG performed during the nystagmus was normal, indicating that the most likely explanation for this periodic nystagmus is metabolic. This form of nystagmus may have resulted from severe hypomagnesemia, possibly associated with thiamine deficiency.     

Two-year-old boy with seizures

Acute traumatic epidural hematomas (EDH) constitute one of the most critical emergencies in neurosurgical management. The rapid spontaneous resolution (<24 h) of EDH is an extremely rare phenomenon. A 17-month-old patient fell from a height of 1.5 m and presented with a 8-mm temporal EDH, an overlying linear skull fracture, and a subgaleal hematoma without evidence of intraparenchymal injury or edema. The patient complained only of mild headache, harbored no neurological deficits, and was, therefore, managed conservatively in the intensive care unit with provision to proceed to surgical decompression in the event of neurological change. A repeat CT study 18 h later revealed near-complete resolution of the EDH with a coincident increase in the volume and spread of the subgaleal hematoma. This is the fourth reported case of rapid spontaneously resolving EDH and the youngest one to date. All 4 cases have coincided with an overlying linear skull fracture. We propose that unlike classical EDH, rapidly resolving EDH in the absence of elevated intracranial pressure (ICP) originates from elevated interstitial pressure in the subgaleal compartment transiently decompressing into the epidural space through a skull fracture and resolving as the pressure in the subgaleal compartment decreases below ICP.     

Medical causes of seizures

Seizures are commonly encountered in patients who do not have epilepsy. Factors that may provoke such seizures include organ failure, electrolyte imbalance, medication and medication withdrawal, and hypersensitive encephalopathy. There is usually one underlying cause, which may be reversible in some patients. A full assessment should be done to rule out primary neurological disease. Treatment of seizures in medically ill patients is aimed at correction of the underlying cause with appropriate short-term anticonvulsant medication. Phenytoin is ineffective in the management of seizures secondary to alcohol withdrawal, and in those due to theophylline or isoniazid toxicity. Control of blood pressure is important in patients with renal failure and seizures. Non-convulsive status epilepticus should be considered in any patient with confusion or coma of unclear cause, and electroencephalography should be done at the earliest opportunity. Most ill patients with secondary seizures do not have epilepsy, and this should be explained to patients and their families. Only those patients with recurrent seizures and uncorrectable predisposing factors need long-term treatment with anticonvulsant medication.     

An islander with seizures

An audit of 690 cases of tuberculosis out of 46,276 patients seen during the last 25 years in a busy general practice is reported. Of the 690 cases, 67% were pulmonary, 33% extra-pulmonary TB. Modes of both types of tuberculosis are described and the reason for increased incidence of extra-pulmonary tuberculosis are discussed.     

Periodic growth in rats

Microknemometry, a novel non-invasive technique, allows the accurate measurements of the lower leg length in the conscious rat, not only daily but even in periods smaller than 24 hours. Its use revealed the presence of nonlinear growth increments (mini-growth spurts) with periods between 4 and 5 days, that presented a gradual decline in amplitude when the animals were getting older, and a maximal growth rate between 0600h and 0900h. A sexual dimorphic growth pattern could be established with females growing less and presenting spurts of lower amplitude and smaller duration than males. High doses of recombinant human Growth Hormone (rhGH) stimulated growth velocity in female rates, but did not show any effect on males. Neonatal Monosodium Glutamate (MSG) treatment reduced growth both in males and females. Growth hormone (GH) replacement therapy in MSG treated animals was capable of increasing growth velocity, from day 30 onwards. The recovery was partial in males and complete in females. In intact male rats growth blockade induced by fasting was not followed by a catch up effect after refeeding, although growth velocity tended to increase and a clear catch up effect on weight was detected. Male rats seemed to grow at a maximal speed over at least the first 60 days of life, that cannot be accelerated with GH treatment, whereas female rats did respond to exogenous GH.     

Audiogenic seizures in curarized mice

Previous experiments have indicated that interference with somatosensory feedback from convulsive movements may lessen the severity of audiogenic seizures in susceptible rodents. For further investigation of this phenomenon, mice were partially immobilized with tubocurarine chloride to attenuate convulsive movements and somatosensory input associated with such movements. In Experiment 1, seizures of mice injected with .15 mg/kg were evaluated behaviorally and compared with seizures of saline-injected litter-mates. The likelihood of clonic-tonic seizures in curarized mice was as high as that of control mice, although convulsive movements were somewhat less violent and seizure fatalities were markedly reduced. In Experiment 2, seizures of mice given .25 mg/kg were evaluated with electroencephalography, and records were compared with those of controls. Despite the near absence of behavioral signs of convulsions, electroencephalograms of curarized mice showed that audiogenic seizures readily occurred. The findings suggest that audiogenic seizures are centrally "programmed" and do not require feedback from convulsive movements. However, it may be possible to disrupt the central "program" by introducing appropriate somatosensory input not normally encountered during audiogenic seizures.     

Psychobiological control of seizures.

Reviews behavioral treatment approaches to controlling epileptic seizures. The various procedures may be classified into 3 major categories: (a) reward and punishment programs, (b) self-control and psychotherapy, and (c) psychophysiological techniques. This review examines the relevant studies and proposes guidelines to eliminate ambiguities and insufficiencies in future studies. It is suggested that behavioral therapies offer substantial promise for the clinical management of seizure problems and that continued activity in this area of behavioral medicine is important for a better understanding of epilepsy and for theory development in both the behavioral and neurological sciences. (80 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Management of seizures and epilepsy

While the evaluation and treatment of patients with seizures or epilepsy is often challenging, modern therapy provides many patients with complete seizure control. After a first seizure, evaluation should focus on excluding an underlying neurologic or medical condition, assessing the relative risk of seizure recurrence and determining whether treatment is indicated. Successful management of patients with recurrent seizures begins with the establishment of an accurate diagnosis of epilepsy syndrome followed by treatment using an appropriate medication in a manner that optimizes efficacy. The goal of therapy is to completely control seizures without producing unacceptable medication side effects. Patients who do not achieve complete seizure control should be referred to an epilepsy specialist, since new medications and surgical treatments offer patients unprecedented options in seizure control.     

Current perspectives on pyridoxine-dependent seizures

An advanced gastric cancer patient with T3N1M0 successfully underwent a curatively total gastrectomy combined with distal pancreatectomy and lymphnode dissection following ELF-P combined chemotherapy. The patient received two courses of etoposide (75 mg/m2, Day 1-5, i.v.), leucovorin (30 mg/body, Day 2-5, i.v.), 5-FU (500 mg/m2, Day 2-5, i.v.) and CDDP (60 mg/m2, Day 1, i.v.). A partial response for the primary lesion and lymphnode metastasis was obtained, and a successful curative resection of the stomach was performed. No drug adverse responses occurred. The effect of ELF-P chemotherapy was confirmed with grade 1b by histopathological examinations. Neoadjuvant chemotherapy with ELF-P may be useful as an inductive approach for advanced gastric cancer.