amenorrhea,hirsutism,obesity

A 32 year old female came with amenorrhea , hirsutism (facial hair) and obesity

a.Give two causes of above complaints.

• Increase cortisol in blood (hypercortisolism)
• Increase prolactin in blood (hyperprolactinemia)

b. State 3 investigation that you would like to do and give reason

• Dexamethasone suppression test

To check the cause of high cortisol , weather it is ACTH dependent or not.

• Skull x-ray

Can visualize if there is pituitary gland adenoma

• Blood test to measure prolaction and cortisol level in blood

High cortisol and prolactin will give rise to above symptoms

On physical examination

Pulse rate : 80 beats/min

Blood Pressure : 150/90 mmHg

There is echymosis.

Dexamethasone test also done.

c.Principal of dexamethasone test

• It has two types, low dose and high dose. Patient will be inject with exogenous steroid (dexamethasone), in low dose-1-2mg and hig dose-8mg. To get the result cortisol level will be measured. A normal result is decrease in cortisol levels upon administration of low-dose dexamethasone.

d.Give the diagnosis

• Pituitary adenoma (which secrete ACTH and Prolactin)

e. Explain pathophysiology of each sign and symptoms in this patient

• hyperprolactinemia-Inhibit effect of gonadotrophin releasing hormone (GnRH) on sex organ-No LH and no FSH-no ovulation and follicular development-amenorrhea
• hypercosticolism-increase fat metabolism-fat redistribution-fat deposited on face , trunk and at the back-obesity
• hypercorticolism-reduce fibroblast activity and collagen synthesis-thin skin-easily bruise-ecchymosis

Extra:

1.In Cushing dz, no change in cortisol on low-dose dexamethasone, but inhibition of cortisol on high-dose dexamethasone. If the cortisol levels are unchanged by low- and high-dose dexamethasone then other causes of Cushing's Syndrome must be considered. In ectopic ACTH secretion (paraneoplastic syndrome), ACTH level will be high even in high dose of dexamethasone.

2.Normally prolactin is inhibited by PIF (dopamine secreted by hypothalamus).

3.Physiologically high prolactin occur in lactation which also will cause amenorrhea.

~living well or living hell? Fighting for future!~

Swelling of face

a.Look at the pictures .what is the gland that causes that abnormalities?

adrenal gland

b.Name the layers at the cortex of the gland and their secretions?

• zona glomerulosa-mineralocorticoid(aldosterone)
• zona fasciculata-glucocorticoid(cortisol)
• zona reticularis-sex hormone(androgen n estrogen)

C.name the abnormalities at these pictures?

• A-moon face
• B-abdominal striae
• C-buffalo hump
• D-acanthosis nigricans

d.In what condition these abnormalities happened?

hypercortisolism

-nisa-

Rheumatoid arthritis

What is Rheumatoid arthritis (RA)?

It is a systemic chronic inflammatory disease affecting multiple tissue but principally attacking the joints, with a peripheral symmetrical non-suppurative arthritis.

Onset: 30-40 y/o

F:M ratio = 3:1

Need to meet 4/7 criteria.

Pneumonic : RF RISES

R : Rheumatoid Factor ( it is not a diagnostic for RA because it can present in other dz
F : Finger/ hand joints involved
R : Rheumatoid nodules ( firm, non tender, oval/rounded mass )
I : Involvement of 3 or more joint area
S : Stiffness in morning => 1 hr
E : Erosions on Xray (xray findings: narrow jt space, erosion of ulnar and radial aspect of PIP jt)
S : Symmetrical arthritis

* Rheumatoid Factor = is an IgM (or can be IgG) autoantibodies directed against Fc portion of IgG




* destruction of tendon, ligament and joint capsules produces characteristics deformities including radial deiation of wrist, ulnar deviatian of finger and flexion-hyperextension of abnormalities of fingers (swan-neck deformity, boutonniere deformity)


Typical clinical manifestation in RA :

• presenting in women of middle age
• begin insidiously, slow development of sign and symptom
• joint inflammation ( pain,swelling, morning stiffness)
• pain gradually relieve on movement
• involving small joint, mostly proximal interphalangeal jt (PIP) and metacarpophlangeal (MCP), distal interphalangeal (DIP) mostly spared
  
• polyarticular
• symmetrical
• nonspecific sign and symptom ( fatigue, malaise and depression )

* reference: xpress revision in short case (pg 126), pathophysiologyof disease (pg 687), basic patho, internet

RHEUMATIC FEVER

Diagnosis: modified Jones criteria
Two major criteria, or one major and two minor criteria, when there is also evidence of a previous strep infection, support the diagnosis of rheumatic fever

Major criteria
The mnemonic JONES is often used to recall the Major Criteria.
1,Joints (Migratory polyarthritis): a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
2.O [imagine heart-shaped O] (carditis): inflammation of the heart muscle which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
3.Nodules (subcutaneous nodules - a form of Aschoff bodies): painless, firm collections of collagen fibers on the back of the wrist, the outside elbow, and the front of the knees. These now occur infrequently.
4.Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spreads outward to form a snakelike ring while clearing in the middle. This rash never starts on the face and is made worse with heat.
5.Sydenham's chorea (St. Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.

an additional way to remember the major criteria is by the mnemonic: C.A.N.C.ER
C: Carditis
A: Arthritis
N: Nodules (sub cutaneous)
C: Chorea
ER: ERythema Marginatum


Minor criteria
1.Fever: temperature elevation
2.Arthralgia: Joint pain without swelling
3.Laboratory abnormalities: increased Erythrocyte sedimentation rate, increased C reactive protein, leukocytosis
4.Electrocardiogram abnormalities: a prolonged PR interval

sources?? encik wikiii..

abnormal hand

a.Describe the picture above

• Thumb is abducted and extended with distal phalanx flexed
• First two fingers are fully extended with a slight flexion of distal phalanges
• Medial two fingers are hyperextended at the MCP joints but flexed at the DIP joints

b.Name this condition

Claw hand

c.what nerve that cause the lesion

ulnar nerve

d.what are the muscles that innervated by this nerve

• FCU,1/2 medial FDP
• small muscle at hand
  all interossei(palmar n dorsal)
  medial 2 lumberical
  hypothenar muscles(Abd DM,FDM,ODM)
  Add pollicis
• Palmaris brevis

e.Pathogenesis of claw hand?

• ulnar nerve innervates the medial 2 of lumbricals
• function lumbrical is flexion at MCP joint and extension of both IP joints(prox n distal)
• therefore,lesion at ulnar nerve results in loss of functinal lumbrical and manifest with its opposite action

thanks to nisa=p

UMN vs LMN lesion

Before proceed, this is clinical diagnostic approach.

1) Which side of body lesion

2) Head involvement or not?

3) Signs of UMN or LMN..

4) At which level the problem arise

Ok! Let make this simple. First, let us make some revision ok. Remember that our motor system is a pathway starts from the motor cortex until Neuromuscular junction.

K. to make things easier. Upper motor cortex has cell body in brain and it’s axon extends into spinal cord and end at SYNAPSE. (so! At this pint kena ingat 2 tract utama iaitu corticospinal tract dan corticobulbar tract. (So it include brain cortex, corona radiate, blab la bla sampai la dia sampai kat anterior horn cell utk synapse ngan LMN)

Lower motor neuron on the other hand has a cell body in spinal cord/brainstem and axon extend outward in a cranial or spinal motor nerve to reach an effector.

For muscle of upper limb and lower limbs, it’s quite easy because remember that corticospinal tract will decussate at medulla to form lateral corticospinal tract. So, lesion of UMN produce contralateral signs. But, still got to remember that there’s also fibres that do not cross and form anterior corticospinal tract. This hence will produce ipsilateral signs. So, at this point, u have to remember the characteristic of UMN LESION which are initial muscle weakness or paralysis, spasticity of affected muscle, hyperactivity of tendon reflex (sebab UMN hantar inhibitory neuron utk reflex. Kalau rosak, dah xde inhibition) and babinsky. In UMN lesion, the wont be any fasciculation, muscle wasting or fasciculation. So, utk LMN lesion, body lesion will be ipsilateral. (ingat kita nyer spinal cord tu. Kan dia nyer nerve yang kuar tu direct je). (tapi ipsilateral boleh jadi juga disebabkan oleh UMN Lesion. So time ni kena la tengok signs dia pulak). Signs of LMN lesion include muscle weakness, wasting, flaccidity, hypoactivity or absent tendon reflex(ingat yg inhibitory neuron x rosak.), fasciculation and no pathological reflex..

Easy right. But for face, it’s quite different due to innervations. Lower face innervate by ipsilateral cerebral hemisphere meanwhile upper face by both cerebral hemisphere. So it can be quite tricky.

Ok. Last step is to determine the site of lesion. Ok! This one you have to memorize the anatomy. Can not help much. But I just know two trick only. First; If face involve, think that the lesion is in your head. Second trick is; if both face and hand involve+ voice production problem(dysphagia or dysphonia) and also sensory impairment always think of lateral medullary symptoms.

K! Gud luck guys…………….