Introduction To Ankylosing Spondylitis

This is a chronic inflammatory arthritis especially affecting sacroiliac joints and spine and characterized by progressive stiffening and fusion of the axial skeleton. It is one of the seronegative spondyloarthropathies.

Incidence

It is most commonly seen in  the age range of 20 to 30 years and male to female ratio is 4: 1 . More than 90% of affected persons carry the histocompatibilty antigenHLA B27

Clinical Signs and Symptoms

Onset is usually insidious. There are recurring episodes of low back pain and stiffness sometimes radiating to the buttocks or thigh. Pain is worse in early morning and after inactivity.

Some patients complian of chest pain aggravated by breathing that is due to involvement of costovertebral joints. There is occasionally heel pain due to plantar fasciitis .

On examination there is failure to obliterate lumbar lordosis on forward flexion. There is pain on sacroilliac compression and tenderness over bony prominenece such as illiac crest, ischial tuberosity and greater trochanter.

There is restriction of movement of lumbar spines in all directions. As the disease progresses stiffness increases throughout the spine.

Associated Clinical findings

  • Iritis occurs in about 25% of patients.
  • Aortic regurgitation, heart blocks and anterior uveitis
  • Pulmonary fibrosis of the upper lobes with progression to cavitation mimicking tuberculosis.

Investigations

  • ESR is often raised.
  • RA factor is absent.
  • HLA B 27 seen in 90% of cases

X Ray Lumbar spine

The sacroilliac joints are eroded with irregular margins and sclerosis of adjacent bone. As the disease advances , the sacroilliac joints may fuse.  Continue reading “Introduction To Ankylosing Spondylitis”

Approach To A Patient With Dysphasia

Dysphasia is a disorder of language and refers to the inability to understand or find words due to a lesion in the dominant (usually left) hemisphere.

Physiology

Wernicke’s area recognizes sound as a language , but a higher concept area is required to convert sound into meaning. This concept area is then connected to Broca’s area where speech is generated. A direct connection , the arcuate fasciculus also exists between Wernicke’s area and Broca’s area.

Types Of Dysphasia

Receptive Dysphasia: The patient is unable to understand; his speech is fluent but words are meaningless. The lesion is in the Wernicke’s area in the temporal lobe.

Expressive Dysphasia: The patient is unable to express despite comprehension. Can cause frustration to patients who know in their minds what to say but cannot get the words out. Due to lesion in the Broca’s area in the frontal lobe.

Transcortical  sensory dysphasia: It is similar to receptive dysphasia but with preserved repetition and is cause by lesion in the parietal-occipital concept area.

Transcortical motor dysphasia: It is similar to expressive dysphasia but with preserved repetition and is caused by incomplete lesion in Broca’s area.

Conductive Dysphasia: Refers to preserved comprehension and output with loss of repetition and is the result of a lesion in the arcuate fasciculus.

Nominal Dysphasia: Means difficulty naming objects and is a result of an angular gyrus lesion.

Examining a patient with Dysphasia

1. Introduce yourself and ask a few simple questions e.g can you tell me your name and date of birth? Continue reading “Approach To A Patient With Dysphasia”

A Brief Discussion On Paget’s disease

Paget’s disease is the disease of the bone caused by a high bone turnover and disorganized osteoid formation. Involved bone is deformed and weak.

How common is paget’s disease

Radiographic evidence is seen in around 2.5% of men over 55 years of age and a little less in women. Less than 10% of those have symptoms. It occurs predominately in people of English origin, including those in North America and Australia. It may be declining in prevalence.

Pathology of bone in  paget’s disease.

Paget’s disease is caused by focal or multifocal areas of  bone resorption by large osteoclasts, followed by increased bone formation, but the new bone is abnormal with a mosaic appearance on microscopy. It is expanded in size, deforms and fractures more easily and is highly vascularised.

Serum and urine biochemical changes seen in paget’s disease

  • Raised alkaline phosphatase.
  • Raised serum osteocalcin ( not routinely used )
  • Raised urinary hydroxyproline and pyridinolone.

Some other causes of raised alkaline phospahtase

  • Hyperparathyroidism
  • Bone metastases
  • Growth
  • Osteomalacia
  • Liver disease

Other investigation that may be useful in paget’s disease

Radiology confirms Paget’s disease by bone expansion. This is rare in metastasis which tend to be sclerotic but expanded. Uncertainty is occasionally resolved by bone biopsy, for example in case of isolated rib expansion. Continue reading “A Brief Discussion On Paget’s disease”

Management Of Patient With GIT Bleeding

A patient with upper or lower GI bleeding after initial evaluation should be managed as follows:

1. Provide venous access with large bore IV (14-18 gauge); central venous line for major bleed and patients with cardiac disease; monitor vital signs, urine output. hematocrit. Gastric lavage is of unproven benefit but  clears the stomach before endoscopy. Iced saline may lyse clots ; room temperature tap water may be preferable . Intubation may be required to protect airway.

2. Type and cross match blood (6 units may be needed for a major bleed).

3. Prepare the surgical team for standby when bleeding is massive.

4. Support blood pressure with isotonic fluids (normal saline); albumin and fresh frozen plasma in cirrhotics. Packed red blood cells when available (whole blood if massive bleeding) ; maintain Hct >25-30.

5. IV calcium (e.g upto 10-20 ml 10% calcium gluconate IV over 10 to 15 minutes) if serum calcium falls due to transfusion of citrated blood.

6. Start empirical drug therapy with antacids , H2 receptor blockers, omeprazole although they are of unproven benefit.

7. In cases of bleeding due to varices give the specific treatment with octreotide, endoscopic sclerosis or band ligation and propranolol as prophylaxis of recurrent bleeding.

 Indications for Emergency Surgery Continue reading “Management Of Patient With GIT Bleeding”

Introduction To Panic Disorder

Panic disorder is an anxiety disorder that is characterized by sudden attacks of fear and panic.  A panic attack may be defined has a sudden, intense experience of fear coupled with an overwhelming feeling of danger and death.

Incidence

It occurs in 1-3 % of the population ; female to male ratio is 2 : 1. Familial aggregation is common. Onset is usually in the second or third decade of life.

Clinical Presentation

Initial presentation is almost always to a non psychiatric doctor , frequently in the ER as a possible heart attack or serious respiratory problem. The disorder is often intially unrecognized or misdiagnosed.

Attacks are sudden, unexpected paroxysms of terror and apprehension with multiple associated somatic symptoms.

Usually reach a peak within 10 minutes , then slowly resolve spontaneously.

Panic disorder is often associated with concomitant major depression.

Diagnostic Criteria for Panic disorder 

The diagnostic criteria for panic disorder requires four or more panic attacks within 4 weeks occurring in non threatening  or non exertional settings and attacks must be accompanied by atleast 4 of the following ;

  • Dyspnea
  • Palpitations
  • Chest pain or Discomfort
  • Feeling of being choked
  • Dizziness/ vertigo/ unsteady feelings
  • Sweating
  • Faintness
  • Trembling.
  • Fear of dying
  • Going crazy or doing something uncontrolled.

Medical Conditions that may mimic Panic attacks

Panic disorders must be differentiated from cardiovascular and respiratory disorders. Conditions that may mimic or worsen panic attacks include

Causes Of Dementia

DementiaDementia may be defined as acquired deterioration in brain’s neuronal function that is characterized by impaired performance of daily living activities including disturbances of memory, thinking, orientation, comprehension, learning and judgement. Memory is the most common cognitive ability lost with dementia.

Dementia has many causes and it is important to identify and exclude the treatable causes.

A list of different causes of dementia is given in a simplified way as follows:

Most Common Causes To Be Listed First

  1. Alzheimer’s disease
  2. Vascular Dementia
  3. Alcoholism
  4. Parkinson’s disease
  5. Drug/Medication intoxication

Causes Listed According To Different Etiologies 

1. Degenerative

  • Alzheimer’s disease
  • Pick’s disease
  • Huntington’s disease
  • Parkinson’s disease
  • Normal pressure Hydrocephalus

2. Vascular

  • Cerebrovascular disease
  • Cranial arteritis
  • Multi infarct dementia
  • Cardiac arrest

3. Traumatic

  • Post head injury
  • Punch drunk syndrome ( seen in Boxers )

4. Intracranial Space Occupying Lesions Continue reading “Causes Of Dementia”

Brief Summary Of Hepatic Encephalopathy

Hepatic encephalopathy which is also known as portosystemic encephalopathy may be defined as a condition in which there is worsening of normal brain functioning as the liver is not able to detoxify the harmful substance in the blood and is characterized by altered level of consciousness and disorientation.

Pathology

The blood bypasses the liver via the collaterals and the toxic metabolities (e.g ammonia, free fatty acids and mercapts ) pass directly to the brain to produce the encephalopathy. There is also an increased sensitivity of CNS neurons to the inhibitory neurotransmittor (GABA) and an increase in circulating levels of endogenous benzodiazepines. Cerebral edema is frequently present and contributes to the development of clinical features.

Precipitating Factors

  • Increased Nitrogen Load
  • Gastrointestinal bleeding
  • Excess dietray protein
  • Azotemia
  • Constipation

Electrolyte and Metabolic Imbalance

  • Hypokalemia
  • Alkalosis
  • Hypoxia
  • Hyponatremia

Drugs

  • Narcotics, Tranquilizers. sedatives
  • Diuretics

Miscellaneous

  • Infection
  • Surgery
  • Portosystemic shunt
  • Superimposed acute liver failure
  • Progressive liver disease

Clinical Manifestations

Symptoms

Disturbance of sleep with sleeping during day and awaking in night is one of the earlies features. Continue reading “Brief Summary Of Hepatic Encephalopathy”

Important Neurological Investigations

There are a large number of neurological disorders and so a number of different investigations are available to help reach the proper diagnosis and then accordingly a proper management.

Here we can discuss a few important ones:

X- Ray Skull

It may show

  • Fracture of skull valut or base.
  • Enlargement or destruction of pituitary fossa due to tumor or raised intracranial pressure.
  • Intracranial calcification due to tuberculoma.

X- Ray Spinal cord

It may show fractures, spondylosis ( degenerative bone disease ) or any destructive bone lesion due to infection or metastasis.

Computed Tomography ( CT Scan )

It is an x-ray slice of 5-10 mm thickness in which different tissues have different densities.

Hypodense tissues ( looking blackish ) 

Infarction, tumor, abscess(having white rim and black center), cerebral edema, encephalitis and resolving hematoma.

CSF and air looks blacker.

Hyperdense tissues ( looking Whitish )

Bone, blood(hemorrhage) and calcifications.

Advantages of CT scan

CT scan easily demonstrates

  • Cerebral tumors
  • Intracerebral hemorrhage and Infarction
  • Subdural and Extradural hematoma
  • Subarachnoid hemorrhage
  • Lateral shift of midline structures
  • Cerebral atrophy
  • Pituitary mass lesion

Limitations of CT scan

Introduction to Von Willebrand’s Disease

Von Willebrand’s disease is the most common congenital disorder of hemostasis. It is transmitted in an autosomal dominant pattern. It is characterized by deficient or defective von willebrand factor. Von willebrand factor plays a role in platelet adhesion to damaged subendothelium as well as stabilizing factor VIII in plasma.

Clinical Manifestations

It can affect both males and females.

Most bleeding is mucosal ( epistaxis, gingival bleeding, menorrhagia and GI bleeding ).

bleeding follows minor trauma or surgery.

Aspirin increases bleeding while pregnancy and estrogen use decreases bleeding.

Investigations

Bleeding time : Increased

PT : normal

APTT : raised

Factor VIII : low

vWF : low

In von willebrand’s disease bleeding time is prolonged while in Hemophilia bleeding time is normal. Continue reading “Introduction to Von Willebrand’s Disease”

Mitral Valve Prolapse

Mitral Valve ProlapseMitral valve bulging back into the left atrium during systole is called mitral valve prolapse. This is also called floppy mitral valve or myxomatous mitral valve.

Etiology

MVP exhibits a stong hereditary componentg and in some patients is transmitted as an autosomal dominant trait.

Second common cause is rheumatic fever mostly seen in developing countries.

It is twice as frequent in women as in men.

MVP may occasionally result from Marfan syndrome, Ehler danlos syndrome and osteogenesis imperfecta.

Pathology

It develops due to myxomatous degeneration of mitral valve in which middle layer of valve leaflet composed of loose, myxomatous material is unusually prominent.

Clinical Features

Symptoms

Most patients with mitral valve prolapse are asymptomatic.

In symptomatic patients it may present as chest pain, dyspnea, fatigue, palpitation, syncopeand even sudden death.

Spontaneous rupture of chordea tendinae may cause a sudden worsening of MR that is hemodynamically severe.

Signs On Examination

On auscultation there is a mid systolic click in mild cases and pansystolic murmur if there is a significant mitral regurgitation. The click is believed to be produced by sudden tensing of the elongated chordae tendineae of prolapsing leaflets.

Thoracic deformities are more prevalent in MVP

BP may be normal or low.

Complications