Giant cell arteritis

You will hear this term over and over again from medical school to MRCP PArt 1 to MRCP PArt 2a to PACES(most of the time in history station for differential of headache or transient blurring of vision).

Mean age of diagnosis 70 years old
Slightly more common in women

Symptoms :
Headache - usually localised
Jaw claudication (pain on chewing)
General malaise
Proximal muscle stiffness and pain
Visual loss (Amaurosis fugax)

Signs :
Tender and thickened scalp veins
Optic disc swollen and later pale (AION)

Investigations :
ESR elevated (one of the differential for ESR >100)
Raised CRP
Raised ALP
Superficial temporal artery biopsy - intimal thickening and medial damage, giant cells with inflammatory cell infiltration in the internal elastic lamina (GOLD STANDARD)
Management :
A general treatment regime for GCA patients that many physicians follow begins with a 70mg/day prednisone dose.

After the first week, the dose is reduced by 10mg every week until the dosage reaches 20mg/day (Schmidt, 2006).

However, some physicians recommend that the high initial dose be maintained for two weeks (Rahman & Rahman, 2005).

Once the 20mg/day level is reached, the dosage is lowered by 2.5mg per week.

Then, when the dosage reaches 10mg/day, it is reduced by 1mg every month depending on the symptoms and test results (Schmidt, 2006).

Most patients remain on corticosteroids for 1-2 years, although some may require long-term steroid treatment (Schmidt, 2006; Piptone et al., 2005).

The reason that treatment of GCA is usually short in duration is because GCA is a self-limiting disease for most patients (although the mechanism for this limitation is unknown) (Azhar et al., 2005; Schmidt, 2006).

Longer corticosteroid treatment is typically required for patients with more severe GCA; these patients usually have cytokine levels (such as TNF-α) above most GCA patients (Hernandez-Rodriguez et al., 2004).

[www.bio.davidson.edu]

ECG Quiz

This is a 63 year old Chinese lady who presented with dizziness.

1. What does the ECG show ?

2. What is the treatment ?

Neurofibromatosis

Station 5 PACES spot diagnosis in skin station ! Remember the criteria of diagnosis, so do not forget to check for Rinne/Weber if there is a tunning fork nearby and also ask for permission to ask about family history.

Table 2 : Criteria for Neurofibromatosis

Type 1 (Von Recklinghausen’s Disease)

Type 2

· Six or more café-au-lait spots, the greatest diameter of which is more than 5 mm in prepubertal patients and more than 15 mm in postpubertal patients · Two or more neurofibromas or one plexiform neurofibroma. Plexiform neurofibroma is considered by some to be a defining lesion of neurofibromatosis type 1 · Frecking in the axilla or inguinal region (Crowe’s sign) · Optic glioma · Two or more Lisch nodules(iris hamartoma) · A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthroses · A parent, sibling or child with neurofibromatosis according to the above criteria

· Bilateral eight cranial nerve palsy confirmed by CT or MRI · A parent, sibling or child with neurofibromatosis type 2 and either unilateral eight nerve mass or any two of the following : neurofibroma, meningioma, glioma, schanoma or juvenile posterior subcapsular lenticular opacity

Aortic Dissection

An interesting case for medical students which I experienced today. I was running the medical clinic when one of the HOs informed me that there is a case needed to be seen in the Echo room.
It was a 64 year old Malay lady who is a known case of hypertension for about 30 years, presented with shortness of breath for 2 days. Clinically she had raised JVP, bibasal crepitations in the lungs and was tachypnoeic. BP 138/80(on antihypertensives) - almost the same the other side. no radio radial or radio femoral pulse.
Basically she had symptoms of congestive cardiac failure.
ECG - sinus tachycardia
CXR - upper lobe divergence, cardiomegaly and widening of the mediastinum
Echo revealed a flap from the ascending aorta to the arch and descending aorta suggestive of Type A aortic dissection EF 40%
She was immediately sent for CT angiogram of thorax and referred to the cardiothoracic.

Discoid lupus

A young lady in station 5 in MRCP or short case in medical students with these skin lesions on the face can only be discoid lupus. As have been mentioned before, discoid lupus is one of the criteria for the diagnosis of SLE. There is atrophy with scarring and hyperpigmented lesions on the face. Do not forget to look at the hair and you will notice scarring alopecia.
I saw this young lady in the combine clinic.

Non medical Books

Well, these would be useful for medical students. If you are interested, pls drop me an email at thienthienlim@gmail.com. I hope I don't need them anymore !

These books are simple to read.

Wallenberg syndrome

A common case but may be underdiagnosed. I was informed by my fellow colleague regarding this interesting case. This patient is a 67 years old Indian man who is a known case of hypertension and diabetes mellitus for the past 10 years. He presented with difficulty in swallowing for the past 2 days associated with unsteady gait.
From the video, you will notice the following :
1) Patient is on a ryles tube and there is hoarseness of voice
2) R Horner's syndrome (miosis and ptosis seen)
3) Lack of movement of the R soft palate causing uvula pulled to the L
4) Reduced pin prick of the R face (inconsistent when I tested for this)
5) Bilateral cerebellar signs
6) Reduced pin prick L half of the body

This patient has R posterior inferior cerebellar infarct (PICA) also known as Wallenberg syndrome. This common stroke is caused by infarction of a wedge of lateral medull lying posterior to the inferior olivary nucleus. Let me try to explain the signs -
1) Nucleus ambiguus and fibers of IXth and Xth CN
2) Descending synpathetic tract
3) Nucleus ambiguus and fibers of IXth and Xth CN
4) Descending tract and nucleus of the V nerve
5) R cerebellar - ?restiform body, cerebellar hemisphere, olivocerebellar fibres, spinocerebellar tract, L cerebellar - patient also had an old infarct at the L cerebellar hemisphere(encephalomalacia from the CT scan)
6) Spinothalamic tract

Thanks to my radiologist, we will proceed for a CT angiogram soon.