OSCE

OSCEDR SUBHASISH DEB

Burdwan Medical College and Hospital

Department of General Medicine

CASE 1

A 42 year old woman was admitted to hospital with

a one month history of progressive forgetfulness,

irritability and confusion. There was no history of

tremor or confabulation. There was no history of

fever, headache, neck-ache or neck stiffness.

Further inquiry revealed she had developed rash

around her neck and in the distal parts of all four

limbs a month prior to the onset of the altered

mental status. The guardians also reported that the

patient had had diarrhea which was watery and had

lasted a week prior to admission. There was no

history of vomiting. She was on Cat 1 for the past 4

months.

Diagnosis?

PELLAGRA (ISONIAZID INDUCED)

Pellagra is due to B3 (niacin) deficiency

Isoniazid induced pellagra is caused by deficiency

of B3 DUE TO a deficiency of B6 (thiamine)

The picture : Casal’s necklace

B6

CAUSES

1. Deficiency of niacin (maize eating population)

2. Deficiency of tryptophan

Decreased intake (meat, fish)

Lucine (inhibits QRPT enzyme)

B6 deficiency (needed by kynunreninase

enzyme)

3. Carcinoid syndrome (conversion to serotonin)

4. Factors causing decreased absorption – Crohn’s

disease, Gasteroenterostomy, chronic alcoholism,

Hartnup’s disease

FEATURES:

4 D’s

Dermatitis

Diarrhoea

Dementia

Death

Others-

Glossitis, loss of appetite, generalized weakness ,

vomiting, abdominal pain.

DUE TO B6 DEFICIENCY:

Microcytic Hypochromic anemia (B6 reqired by

delta ALA – 1st enzyme of heme sys)

Seizures :

glutamate

glutamate decarboxylase B6

GABA

Hyperactivity of neurons due to excess of glutamate

Homocysteinuria :

Cystathianone B synthase requires B6 (PLP) to convert

homocysteine to cystathione – increased CVA chances

TREATMENT

Always give Pyridoxine with isoniazid

B3 deficincy treated by:

oral nicotinamide (niacin) 100-200mg TDS x 5days

Adverse effects of niacin: FLUSHING

Due to tachyphylaxsis

Premedication with ASPIRIN

Niacin combined with

LAROPIPRANT a

prostaglandin D2 receptor 1

antagonist

CASE 2

A 58 year old woman came with a chief

complaint of syncope. She also had low

grade fever 38C.

Her 12 lead ecg showed..

BRUGADA SYNDROME

First described in 1992 by Pedro and Josepg

Brugada

Associated with sudden cardiac death

Individuals are usually healthy with structurally

normal hearts

Generally considered a hereditary disease

More common cause of sudden cardiac death than

previously recognized

HOW COMMON IS IT??

Responsible for up to 20% of sudden deaths in pts

without any structural heart abnormality

Responsible for 4-5% of all sudden deaths

Incidence varies in different populations

Most common in young males

First onset of symptoms (syncope. Sudden death) ~

40 yrs

BRUGADA SYNDROME

Mortality ~10% per year if not treated with internal

cardioverter-defibrillator (ICD)

Antidsyrhythmics have no effects on prognosis

Syndrome characterized by:

ECG abnls. in lead v1-v3

Polymorphic or monomorphic VT

Structurally normal heart

Familial occurrence in ~ half of the pts

BRUGADA SYNDROME

ECG findings in v1-v3: RBBB or IRBBB

ST segment elevation --- 2 types

“Coved-type” – most common

“Saddle-type”

Findings can vary depending on may factors including

fever/ambient temp

Definitive diagnosis: EPS (electrophysiology study

in EP labs)

Saddle

typeCove

type

TYPES:1. Type 1 :

Coved ST segment

J point elevation with ST segment elevation >=0.2mV

Negative T wave

2. Type 2:

Saddle back configuration of ST

High take off of ST >0.2mV

Ending in positive or biphasic t wave without touching base

line

3. Type 3:

ST elevation <0.1 with either of the morphologies

UNCOMPLICATED RBBB

ST depression

CASE 3

Papules extending to form a yellow–red plaque

covered with telangiectatic vessels on the

patient's forearm. CBG 330. ???

NECROBIOSIS LIPOIDICA

DIABETICORUM

Necrobiosis lipoidica was first described by

Oppenheim in 1929 as a chronic granulomatous

dermatitis of unknown cause.

female:male ratio of 3:1

Mostly associated with Type 1 DM

FEATURE:

initially presents with well-circumscribed

erythematous papules, which develop into large,

irregularly delineated plaques with a waxy, yellow center

the epidermis becomes thin and transparent, allowing underlying vasculature to become visible

The involved peripheral tissue is slightly raised and has a reddish-blue color

Pathophysiology Exact cause not known

an inflammatory disorder characterised by collagen

degeneration, combined with a granulomatous response

Diagnosis Skin biopsy

demonstrating superficial and deep perivascular and

interstitial mixed inflammatory cell infiltrate

necrotising vasculitis with adjacent necrobiosis and

necrosis of adnexal structures

Presence of lipid in necrobiotic areas may be

demonstrated by Sudan stains

No clearly defined cure.

CASE 4

A 18yr old boy came with deafness to the ENT opd. The

new female resident finds it to be SNHL and refers him

to MOPD. The physician asked for an MRI and saw this

plate (Fig 1)

The physician referred the pt to SOPD for a biopsy. After

having completed the up hill task of getting an aesthetic

fitness, finally the pathologist in his exam found

VEROCAY bodies in the specimen and told to correlate

clinically!

The surgeon meanwhile didn’t understand much as

usual and send the pt back to MOPD.

The physician gave a diagnosis of__________ and send

the pt back to the surgeon for surgical treatment.

NEUROFIBROMATOSIS 2 A/k MISME syndrome- Multiple Inherited

Schwannomas, meningiomas and ependymomas

Less common than NF-1

Due to mutation of merlin (a/k schwannomin) in ch

22q12 , AD

Symptoms generally occur at late teen to 20yrs

CLINICAL FEATURES:

Hallmark of NF2 is hearing loss due to vestibular

schwannoma

Others:

Headache

Balance problems and peripheral vertigo

Facial weakness- compression of VII nerve

Deafness and tinnitus

Other brain and spinal tumours

DIAGNOSIS:

Confirmed diagnosis:

bilateral vestibular schwannomas (may also be known

as acoustic neuroma)

Probable diagnosis:

family history of NF2 AND unilateral vestibular schwannomas or any 2 of the

following tumor types: meningioma, glioma,

schwannoma, juvenile posterior subcapsular lenticular

opacity, juvenile cortical cataract

TREATMENT

Surgery is the primary treatment for most peripheral

nerve tumors associated with NF2.

Systemic medical treatment:

Bevacizumab (still in trial)

Management of hearing loss:

Cochlear implant, ABI- auditory brain stem implant

For meningioma;

Sunitinib (in trial)

For vestibular schwannoma:

Lapatinib (in trial)

THANK YOU