Interesting case - 7.28.2011

For numerous reasons, it's been awhile since I posted a case... Here's a case that I had when I was a first year path resident. It's near and dear to my heart, so I held on to it for awhile.

This patient was a 46-year-old female with a several month history of rapidly-progressive cognitive decline.  Neuroimaging demonstrated numerous foci of white-matter T2 and FLAIR signal abnormalities scattered throughout the cerebral hemispheres; gadolinium administration revealed numerous, ill-defined small foci of contrast enhancement. The antemortem clinical differential diagnosis included nearly all known causes of human disease, including infection, ADEM, AHLE, metastatic carcinoma (remote history of breast carcinoma). Despite aggressive clinical interventions, the patient expired after a several-week stay in the hospital.

Sectioning of the brain demonstrated (sadly, images not available) irregular, ill-defined areas of softening and hemorrhage in the hemispheric white matter. In areas, the gray-white junction was effaced.

Low power (H&E); frontal lobe

Low power (LFB); frontal lobe, white matter

High power (H&E); frontal lobe

High power (H&E); frontal lobe

High power (H&E); frontal lobe, neocortex

High power (H&E); high power, frontal lobe leptomeninges

No hints needed here. This case qualifies as an "Aunt Minnie". If you've seen it before, the diagnosis is unquestionable. If not, good luck...

Click here for the diagnosis...

Interesting case - 7.1.2011

Unfortunately, all vacations must come to an end. The AANP meeting in Seattle went quite well, and did a good job of highlighting the potential diagnostic pitfalls of muscle biopsies (i.e. scared a lot of people). It was really nice to get back home though. I just finished "checking out" at UT Southwestern, so I am no longer employed by UT Southwestern. Next up, I start my new job in Southern California on Tuesday. And so the adventure continues...

On to the case. I've been holding on to this case for quite awhile, as I didn't want to spoil the presentation of the case at the AANP diagnostic slide session. Now that is over, I can share what I thought was a fantastic case. This patient was an adolescent female with an 11-year history of tremor, gait instability, dysarthria, tongue fasiculations, and temperature instability. The immediate preterminal period was characterized by disabling dyskinesias, choking and aspiration, and progressive cognitive decline.

Gross brain; vertex

Coronal section, level of nucleus accumbens

Cross sections, brain stem and cerebellum

Substantia nigra, low-power; H&E

Subthalamic nucleus; H&E

Anterior horn, spinal cord; H&E

Cerebellar cortex; H&E

Substantia nigra; H&E

This is a classic "Aunt Minnie" diagnosis. If you've seen if before, the diagnosis is not difficult. If not, head scratching and shuffling pages ensues... Never fear, helpful immunostains will be provided!

Click here for the diagnosis...