Vikram Singhal

Successful treatment of vincristine induced ptosis and polyneuropathy with pyridoxine and pyridostigmine in a child with acute lymphoblastic leukemia

Vincristine is used in the treatment of solid tumors, lymphoma and leukemia in children. The dose-limiting toxicity is its neurotoxicity. We describe a 2-year-old girl with acute lymphoblastic leukemia who developed vincristine-induced polyneuropathy with bilateral ptosis and recovered on treatment with pyridoxine and pyridostigmine.

Guillain–Barre syndrome with hyperreflexia: A variant

Guillain–Barre syndrome (GBS) is a common cause of acute peripheral neuropathy and is characterized by hyporeflexia or areflexia. Hyperreflexia has been rarely reported with acute motor axonal neuropathy. A 10-year-old boy presented with asymmetrical weakness of upper and lower limbs and change of voice. Weakness progressed in the hospital with involvement of multiple cranial nerves, preserved deep tendon jerks with extensor plantar, and normal abdominal reflexes. He was treated with IV immunoglobulin and IV methylprednisolone. He was able to walk with support with normal voice at the time of discharge. GBS should be a differential diagnosis in patients with acute quadriparesis even if there are preserved deep tendon reflexes.

Mediastinal enteric cyst in a neonate

Mediastinal enteric cysts are relatively uncommon, and patients tend to present at a later age compared to those with duplications in other areas of alimentary canal. The tendency of enteric cyst to enlarge and produce airway obstruction is sufficient reason for early surgical removal. We report on a case of mediastinal enteric cyst in a neonate with respiratory distress for its early presentation and management. The embryological basis and anatomical issues relating to duplication cysts of the gastrointestinal tract is discussed.

Tuberous sclerosis with rhabdomyoma.

Tuberous sclerosis is a neurocutaneous syndrome characterized by abnormalities of both the integument and central nervous system. We present a case of tuberous sclerosis with rhabdomyoma in the heart. This was a 1½-year-old female child with infantile spasms and rhabdomyoma in heart with mother having neurocutaneous markers of tuberous sclerosis. Magnetic resonance imaging brain and electroencephalography findings were consistent with diagnosis.

Tuberculous osteomyelitis in Job syndrome

Job syndrome (JS) is a rare primary immunodeficiency disorder characterized by the triad of raised serum immunoglobulin E levels, recurrent skin and pulmonary infections. A 6-year-old boy presented with the characteristic triad of JS and left leg swelling, which on evaluation was found to be tuberculous osteomyelitis. JS being an immunocompromised state can predispose to tuberculous infections and one has to be vigilant enough for early diagnosis and treatment to prevent deformity.

Klippel-Trenaunay syndrome and type 1 neurofibromatosis: A chance association?

Klippel-Trenaunay syndrome and neurofibroma type I (NF1) are rare disorders with cutaneous and neurological features and not reported to be associated together. We report an 11-year-old female child who presented with extensive skin lesions, giant hemangioma and left lower limb hypertrophy. Her father had features of NF1. We report this case because of its rarity and need for long-term follow-up.