Nitin Patel

The use of levetiracetam in refractory status epilepticus

Six patients with status epilepticus (SE) of various etiologies refractory to at least two antiepileptic drugs (AEDs) had complete cessation of their seizures following administration of oral levetiracetam (LEV). Seizure types included convulsive, focal, and nonconvulsive status epilepticus. Effective doses of levetiracetam ranged from 500 to 3000 mg/day, achieving seizure control within 12-96 h. No significant adverse events were noted. Adjunctive levetiracetam should be considered for patients with status epilepticus unresponsive to initial therapy.

Dejerine-Sottas neuropathy in mother and son with same point mutation of PMP22 gene

We studied a 25‐year‐old black woman with healthy parents and her 2‐year, 11‐month‐old son. Her motor development was delayed and she started to walk with support when she was 6 years old. She never walked independently and had always used a wheelchair. Neurological evaluation showed severe weakness and atrophy of her feet, legs, and hands, bilateral pes cavus and hammertoes, corrected scoliosis, hypesthesia for proprioception and vibration sense in both feet and ankles, and areflexia. She had normal intelligence. Her son also had delayed motor milestones and was still unable to stand and walk independently at almost 3 years. Neurological evaluation revealed diffuse muscle hypotonia and weakness with generalized areflexia and normal intelligence. No muscle atrophies or feet deformities were noticed. Nerve conduction velocities showed significant slowing (less than 5 m/s) with prolonged distal latencies (above 30 ms). Compound motor action potential amplitudes were markedly reduced. Electromyography revealed polyphasic motor unit potentials. Molecular genetic studies indicated a Trembler type missense point mutation of exon 4 of the peripheral myelin protein 22 gene that led to the substitution of a spartic acid for glycine in both the mother and her son. Her parents showed normal DNA studies.

Development of tics in a thirteen-year-old male following atomoxetine use.

Tics and Tourette syndrome are common comorbidities of patients diagnosed with attention-deficit/hyperactivity disorder (ADHD). One of the mainstay pharmacologic therapies for ADHD has been stimulants. However, this class of drugs has been associated with tic exacerbations, thus limiting their utility in this patients subgroup. Atomoxetine has been explored as an alternative treatment as one of the few non-stimulants available to treat ADHD. Early data identifies atomoxetines influence on Tourette symptomatology to be not merely equivocal but potentially suppressive in the manifestation of tics. There are, however, case studies describing patients experiencing recurrences of tics following treatment with atomoxetine. We present a unique case of a patient, without any prior history of a movement disorder, who developed tics following a single dose of atomoxetine that did not improve until interventional therapy was initiated.

Central hyperventilation related to administration of topiramate

Topiramate is a recently released antiepileptic agent used in the treatment of patients with refractory seizure disorders. In addition to its antiepileptogenic activities, it results in inhibition of carbonic anhydrase isoenzymes II and IV, which are present in the central nervous system. A 15-year-old female who presented with hyperpnea and primary respiratory alkalosis is reported. Other possible etiologies of the central hyperventilation syndrome were excluded. The problem resolved within 24 hours after discontinuing topiramate.

Childhood-onset multiple sclerosis and mood disorders: a case study.

Multiple Sclerosis (MS) is rare in children. Little research exists regarding emotional and behavioral disorders in childhood-onset MS, despite the occurrence of such problems in adults with MS. This paper describes the cognitive and behavioral characteristics of a boy diagnosed with MS at age 9 and mood disorder at age 10. He displayed no cognitive or behavioral problems prior to the onset of physical symptoms of MS. Three years after diagnosis, this child showed persistent problems with speed of processing, visual-motor skills, and parent and teacher-reported executive functioning. In addition, he had difficulties with emotional lability, behavioral disinhibition, depression, and social interaction. As with adults, children with MS may be at increased risk for mood disorder compared to their peers. Mood disorders in children with MS are likely to be multiply determined, although the specific causal mechanisms are unknown.

Peripheral neurophysiology of acute distal spinal cord infarction

F-wave abnormalities, in the presence of normal distal motor nerve conduction, most often are the first indicators of proximal peripheral nerve dysfunction in demyelinating polyradiculoneuropathies. However, a 15-year-old female-who developed lumbosacral spinal cord infarction with paraplegia, sensory loss, and incontinence beginning 15 hours after a fall-studied electrophysiologically at 2 days postparaplegia manifested absent lower-extremity f-waves and H-reflexes and normal compound muscle action potentials and distal motor and sensory conduction velocities. Subsequent evaluations demonstrated permanent loss of compound muscle action potentials, f-waves, and H-reflexes and prominent acute denervation in paralyzed lower-extremity muscles. Thus early f-wave and H-reflex loss can also occur in spinal cord disease, thereby representing the first evidence of motoneuron destruction.

Hemobilia Secondary to Transjugular Intrahepatic Portosystemic Shunt Procedure: A Case Report

A 59 year-old woman with liver cirrhosis due to hepatitis C, complicated by refractory hepatic hydrothorax was treated with a TIPS (transjugular intrahepatic portosystemic shunt) procedure. The procedure was complicated by substantial gastrointestinal hemorrhage. EGD (esophagogastroduodenoscopy) was performed and revealed hemobilia. A hepatic angiogram was then performed revealing a fistulous tract between a branch of the hepatic artery and biliary tree. Bleeding was successfully stopped by embolization of the bleeding branch of the right hepatic artery. Hemobilia is a rare cause of upper gastrointestinal bleeding with an increasing incidence due to the widespread use of invasive hepatobiliary procedures. Hemobilia is an especially uncommon complication of TIPS procedures. We recommend that in cases of hemobilia after TIPS placement, a physician should immediately evaluate the bleeding to exclude an arterio-biliary fistula.

Metastatic prostate cancer to the duodenum: A rare case

Prostate cancer is the third most common cancer in man. About 1 in 6 males developed prostate cancer and 1 in 35 males die of this disease. Prostate cancer behavior ranges from microscopic tumors to aggressive cancer with metastatic potential. While metastasis to bone is relatively common, prostate cancer rarely metastasizes to the cecum, pituitary gland, small bowel, maxillary sinus and skin. Our case report presents a rare presentation of metastatic prostate cancer to the duodenum. Our search of the literature found only 2 cases of prostate metastases to duodenum published from 1966 to the present. To our knowledge this is the third case of metastatic prostate cancer presenting with duodenal metastasis. Although it is rare but in symptomatic patients small intestine metastasis should not be ignored with advanced prostate cancer. The case demonstrates a novel presentation of a common malignancy, and should raise awareness in clinicians and radiologists that prostate cancer can present with distant metastases in absence of any local lymphadenopathy.

Intravenous Glucagon Beneficial During Colonoscopy in Patient with IBS

Background and Aim: Irritable bowel syndrome (IBS) is a functional bowel disorder characterized by abdominal pain or discomfort and altered bowel habits. Patients with IBS requiring colonoscopy take longer time to cecum with higher need for medications used for conscious sedation. Glucagon is routinely used during endoscopic procedures to reduce peristalsis that interfere with the procedure. However, randomized controlled data using glucagon during endoscopic procedures are lacking. We designed a prospective randomized placebo-controlled trial to study the effect of intravenous glucagon given during colonoscopy. Materials and Methods: We received approval from the FDA for this off-label use of glucagon during colonoscopy. This is a double-blind randomized placebo-controlled study. Patients were selected based on ROME III criteria for IBS; patients who met Rome III criteria and had an indication for colonoscopy for age-specific colon cancer screening or for work up of any alarm signs. We selected 34 patients meeting the Rome III for IBS and randomized into Group A and Group B. Both the performing endoscopist and patients were blinded. These patients in both groups initially received a standard dose of conscious sedation, up to 100 mcg of fentanyl and up to 5 mg of midazolam intravenously. In Group A, 17 patients, in addition to conscious sedation, received 1 ml saline as placebo. In Group B, 17 patients, in addition to conscious sedation, received 1 mg of intravenous glucagon. Parameters evaluated were as follows: 1) Total time required for colonoscopy 2) Completion of colonoscopy as documented by cecal intubation or visualization of appendicular orifice 3) Level of comfort in patient concerned to postprocedure spasmodic pain, which was based on Wong-Baker FACES pain rating scale and 4) Calculate the amount of sedation required in both groups of patients and also at what extent glucagon helped to decrease the requirement of sedatives. Data was analyzed using the student t-test.

Venous Sinus Thrombosis and Consumptive Coagulopathy: A Role for Heparin?

Cerebral venous sinus thrombosis is a relatively rare but serious condition, more commonly affecting children and pregnant women. It can be precipitated by dehydration. Despite the frequent coexistence of hemorrhage in venous infarcts of patients, clinical trials in adults recommended the use of anticoagulation. No randomized, clinical trials exist in the pediatric age group. Rarely, consumptive coagulopathy is reported to coexist with cerebral venous sinus thrombosis. We report on a child with venous sinus thrombosis and consumptive coagulopathy developing after routine tonsillectomy and its successful management with anticoagulation.