Saturday, February 27, 2010

Creutzfeldt-Jakob disease (CJD)

Creutzfeldt-Jakob disease, short-formed as CJD, is a nervous system disorder caused by prion (a kind of virus). Pathology slide of a brain infected by this disease is typically show spongiform encephalopathy, or what was called "bubble and holes".1

Of course taking a slide from a dead body is not something practical to do in terms of diagnosing a patient. Therefore, we should look for signs and symptoms as well as using a correct diagnostic tool to diagnose a case of CJD.

Just to provide a case from website about this disease: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2626364/
http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease

One thing I want to point out is that this disease can cause dementia. The guy can be normal, and you can confuse patient with CJD to Parkinson, like I do. Looking for signs and symptoms as the presence of myoclonus. As such, one should look for a possibility of CJD since myoclonus isn't a part of Parkinson's disease. One characteristic diagnosis we can do is an electroencephalogram showing periodic high amplitude sharp wave http://www.websciences.org/cftemplate/NAPS/archives/indiv.cfm?ID=20060373 As quoted: In sporadic CJD (sCJD), the EEG exhibits characteristic changes depending on the stage of the disease, ranging from nonspecific findings such as diffuse slowing and frontal rhythmic delta activity (FIRDA) in early stages to disease-typical periodic sharp wave complexes (PSWC) in middle and late stages to areactive coma traces or even alpha coma in preterminal EEG recordings.2

The disease can be deadly and there is no treatment for this disease.


1. Edward F. Goljan Rapid Review Pathology 2nd edi. Mosby Elsevier. 2007; 578-579
2. WIESER HG, SCHINDLER K, ZUMSTEG D. Clin Neurophysiol. 2006 [cited 2006 Jan 24] Available from: http://www.websciences.org/cftemplate/NAPS/archives/indiv.cfm?ID=20060373

Friday, February 26, 2010

Foville's syndrome

Category: Neuroanatomy.

When one half of the pons is injured involving the corticospinal tract (above the decussation of the pyramids), the facial nerve (CN VII) nucleus and/or facial nerve fibers, and the nucleus of CN VI (abducens) or the nearby paramedian pontine reticular formation (PPRF) fibers to CN VI, the resulting constellation of signs/symptoms includes: contralateral spastic weakness/paralysis (weakness, hypertonia, hyperreflexia and Babinski's sign), ipsilateral upper and lower facial weakness and loss of volitional abduction of the ipsilateral eye (horizontal gaze paresis).

From: http://en.wikipedia.org/wiki/Foville's_syndrome
Foville's syndrome is caused by the blockage of the perforating branches of the basilar artery in the region of the brainstem known as the pons.[1]
Structures affected by the infarct are the PPRF, nuclei of cranial nerves VI and VII, corticospinal tract, medial lemniscus, and the medial longitudinal fasciculus.

The syndrome is reported in http://www.nzma.org.nz/journal/119-1232/1928/ in which the case mentioned the syndrome on a suspected Wernicke’s encephalopathy.

There is another recorded case: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1143046/