Tuesday, March 25, 2008

Impulse control and PD Quick hits AAN 2008

1. Patients with PD showed altered decision making under ambiguity but had preserved decision making under risk conditions with explicit rules for gains and loss.
2. Acamprosate was used to treat impulse control disorders in 2 patients with PD in whom discontinuation of dopaminergic drugs did not help. It worked
3. Topiramate was used in 8 Spanish patients to reduce ICD and it appeared to work.
4. The ICD spectrum included pathological gambling, hypersexuality, shopping, computer use and binge craving of sweets, which should be specifically sought in the history taking.

AAN 2010
Hoarding may be a component of the syndrome

Neuroopthalmic findings that reliably differentiate PSP and PD

P05.042 AAN book 2008 Brad Katz et al. Salt Lake City Utah

In addition to standard criteria , the following differentiated the 2 disorders: slowed horizontal saccades, square wave jerks, eyelid opening apraxia, abnormal vertical OKN's, lid retraction stare, blink rate and light sensitivity were all implicated. A cutoff score of 2 or more of the the following three features: greater than 5 swj's, abnormal OKN's downward, and lid retraction stare identified all of 28 patients correctly (including placebos).

Hardwick A, Rucker JC, Cohen ML, et al.  Evolution of oculomotor and clinical findings in autopsy proven Richardson syndrome.  Neurology 73; 2122-2123 2009.

Authors note the SPEED of vertical saccades (more than amplitude) remain the cornerstone for differentiating PSP from other disorders.  Clinical presentation was young, absent falls, complaints of horizontal diplopia due to vergence abnormalities, cognition c/w FTD.  At autopsy atrophy was restricted to the superior colliculi with rare NFTs.

Hemiparkinson-hemiatrophy syndrome

Wijemann S, Jankovic J. Hemiparkinsonism-hemiatrophy syndrome. Neurology 2007; 69: 1585-1594.

Seminal description was made by Klawans in 1981 (Neurology) with hemiatrophy beginning in childhood and hemiParkinsons beginning in early adulthood (before 45). The hemiatrophy may be unnoticed. Symptoms usually begin ipsilateral to the hemiplegia but may spread to the other side. The condition may progress slowly and respond variably to levodopa. This series of 30 patients is the largest to date published. Patients may present initially with dystonia, that is often action induced and may involve the leg or arm only, induced by walking, running, or writing. There was no right or left sided predominance. Patients could present however, with early morning dystonia, tremor, bradykinesia, or gait imbalance. Patients often had scapular winging, raised shoulder , unequal leg length, brisk reflexes and extensor plantars. Occassionally patients had problems in pregnancy or early development especially walking. Scoliosis was common. MRI findings include asymmetric lateral ventricles, volume loss, thalamic or arachnoid cyst. Unilateral changes including calvarial thickening, expansion of the ethmoid, frontal or mastoid sinus, and elevation of the petrous ridge and greater wing of the sphenoid as in the Dyke Davidoff Mason syndrome are reported.

Negative signs and symptoms, that is thsoe infrequently or not seen, include axial signs such as swallowing or speech difficulty or symmetric freezing (although one patient had a relatively unusual sign of unilateral freezing).

The hemiatrophy involved, in order of frequency, hands, feet and face. Some patients first noted difficulty fitting shoes. Its developmental character differentiates it from atrophic conditions such as the Parry Romber syndrome or linear scleroderma. Perinatal injuries are not universal and some cases are traumatic, such as a patient who suffered a gunshot wound to the head at age 6.

Genetic mutations such as the parkin mutation are reported, albeit rarely.

Surgery has been used including VIM DBS, bilateral STN DBS

The authors speculate that patients might have less dopaminergic neurons at birth, perhaps due to a toxin such as lipopolysaccharide, paraquat, or fungicide maneb.

Friday, March 07, 2008

Tegretol and Asians

before giving screen for HLA B1502

FDA warning for rash risk: http://www.fda.gov/cder/drug/InfoSheets/HCP/carbamazepineHCP.htm

Thursday, March 06, 2008

tamiflu and delirium

Tamiflu Label Updated with Neuropsychiatric Warning
The FDA and Roche Laboratories have revised the product label for Tamiflu (oseltamivir phosphate) to include a warning on possible neuropsychiatric events. The updated label is based on recommendations from the agency's Pediatric Advisory Committee meeting in November 2007.
Postmarketing reports indicate that some patients with influenza who were receiving Tamiflu had delirium and abnormal behavior leading to injury and even death. Most of the cases occurred in children and in Japan.
Although it's not clear whether Tamiflu caused these events, the label cautions clinicians to monitor their patients for abnormal behavior when taking the drug. Adverse events should be reported through the FDA's MedWatch site.
FDA alert (Free)
Tamiflu prescribing information (Free PDF)
FDA's MedWatch site (Free)