Cluster headache

Vignette says a 35 year old male presents to the neurology clinic with severe, episodic, unilateral periorbital pain, that started 48 hours ago; The pain is excruciating, stabbing in nature and lasts around 30-90 minutes; He also reports of having ipsilateral eye reddening with lacrimation, nasal stuffiness/rhinorrhea, ptosis and miosis during these attacks; He has had multiple episodes daily, often occurring at night, waking him from sleep; He reports of having similar headaches in the past, occuring in cycles that last for weeks to months (cluster periods), followed by months of remission; MRI of brain is normal; Diagnosis? 

Diagnosis is Cluster headache.

Clinical Features:-
1. Severe episodic unilateral periorbital (intense/sharp) pain; attacks lasting 15 minutes to 3 hours with spontaneous remission; worsened by alcohol and during night; men being 10 times affected as compared to women.
2. P/E shows autonomic symptoms (e.g. ipsilateral horner syndrome; nasal stuffiness/rhinorrhea; eye reddening with lacrimation).

Treatment:-
A. Abortive therapy:- 
1. 100% of oxygen(1st line agents).
2. Triptans (e.g. intranasal sumatriptan 20 mg/ subcutaneous sumatriptan 20 mg) or Ergotamines.
B. Preventive therapy(Prophylaxis):- Verapamil (1st line therapy); Corticosteroids, Sodium valproate, Lithium (2nd line therapy).

Gilbert syndrome

Vignette says a 20 year old male presents to his primary care physician with chief complaints of yellowing of his eyes over the past few days; There is no significant past medical history; He reports that he has been under a lot of stress recently as his finals are coming near; Vital signs are stable; Examination shows scleral icterus; Laboratory studies show normal CBC, normal AST & ALT, total bilirubin of 2.4 mg/dL (normal range is 0.1-1.2 mg/dL) and direct bilirubin of 0.2 mg/dL (normal range is less than 0.3mg/dL); Direct coombs test is negative; Diagnosis?
 
Diagnosis is Gilbert syndrome.

Gilbert syndrome is the most common inherited metabolic disorder of bilirubin which results from the partial deficiency UDP glucuronosyltransferase activity.

Presents with jaundice and elevation of unconjugated bilirubin following stress (e.g. fever, fasting, dehydration, vigorous exercise, menstruation, surgery, trauma).

Diagnosis:-
1. Elevated unconjugated bilirubin (i.e. indirect hyperbilirubinemia).
2. CBC, PBS, RFT and LFT are normal.

Treatment:- Gilbert syndrome is a benign condition and does not require treatment.

Intussusception

Vignette says a 24 month old male child presents to the emergency department with chief complaints of intermittent episodes of abdominal pain, and multiple episodes of vomiting over the past 8 hours; The abdominal pain is severe and crampy in nature lasting for few minutes, which resolves spontaneously and recur every 15-30 minutes, during the pain the child draws his knees to his chest and cries intensely; His mother also noticed reddish mucus like stool in diaper over the past hours; His mother notes that the child had acute gastroenteritis 7 days back and was managed with ORS and probiotics at home; The child is irritable and anxious; Abdominal examination shows soft, mild distended abdomen with sausage shaped mass on palpation in right upper quadrant; Abdominal ultrasonography shows target sign; Diagnosis? 

Diagnosis is Intussusception.
 
Intussusception is telescoping of one portion of the intestine into the other.

Etiologies are meckel’s diverticulum, hypertrophy of payers’ patches, and mass.

Presents with severe colicky abdominal pain, vomiting, bloody stool, right hypochondrium sausage shaped mass on palpation and emptiness in the right lower quadrant (Dance sign); The child draws the legs up to the abdomen during the episode of abdominal pain.
 
Diagnosis:-
1. Abdominal x-ray (i.e. erect and supine) shows signs of bowel obstruction. 
2. USG shows target sign or Bull’s eye sign. 
3. Air enema is both diagnostic and therapeutic.
 
Management:-
1. Air enema is both diagnostic and therapeutic.
2. Surgical reduction if air enema fails, signs of peritonitis or perforation presentation.