Pituitary Apoplexy

Pituitary apoplexy occurs when a pituitary tumor spontaneously hemorrhages or when it outgrows its blood supply. Pituitary adenomas can suddenly bleed internally, leading to an abrupt increase in size which can cause compression of the pituitary gland and perisellar structures, the optic nerves, and the nerves that control eye movements. Pituitary tumor apoplexy is a rare endocrine emergency and is a potentially life-threatening disorder. All types of pituritary adenomas can be associated with apoplexy, especially larger tumors (macroadenomas). Signs and symptoms of pituitary apoplexy include headache, visual impairment, nausea and vomiting, altered mental status, meningismus, and fever. Headaches are the most prominent symptom in patients with pituitary apoplexy.

Pituitary apoplexy is best diagnosed with an MRI scan of the brain, with special attention to the pituitary. A CT scan of the pituitary gland also shows and abnormality but MRI is the preferred exam.

Effective treatment of pituitary apoplexy requires prompt administration of high-dose corticosteroid along with careful monitoring of fluid and electrolyte levels, and in most cases urgent transsphenoidal surgery is required. Careful monitoring of hormonal status by an endocrinologist during the acute phase of apoplexy and in the recovery phase is essential. Most patients have significant visual improvement after surgery, however allot of patients will have permanent hormone deficiencies due to pituitary injury and will require hormone replacement therapy for life. Thanks to the steroid medications we have today, patients rarely die from pituitary apoplexy if promptly diagnosed and treated.

Labyrinthitis

Labyrinthitis is an ear disorder that involves irritation and swelling of the inner ear. The parts of the inner ear become irritated and this interferes with their functions in hearing and balance. There are many causes of of labyrinthitis the most common cause occurs after an ear infection or upper respiratory infection. Symptoms include vertigo, dizziness, loss of balance and equilibrium, nausea and vomiting, ringing of the ear, and difficulty focusing the eyes because of involuntary eye movement. There are many different names for Labyrinthitis such as:
-Bacterial labyrinthitis
-Serous labyrinthitis
-Neuronitis
-Viral neurolabyrinthitis

Labyrinthitis usually goes away within a few weeks. Some symptoms may need treatment with antibiotics. Most patients are completely better and their hearing returns to normal. However, calcifications or ossificans can appear after Labyrinthitis.

Labyrinthitis ossificans. Axial CT shows new bone formation partially obliterating left cochlea.

Labryinthitis ossificans is the pathologic formation of new bone within the lumen of the otic capsule and usually occurs in response to a destructive or inflammatory process. It is a condition in which the inner ear is filled with fibrotic tissue, which then calcifies. It mostly affects the cochlea, but the vestibule and semicircular canals can also be involved. Labyrinthitis ossificans most commonly develop after having bacterial meningitis. Labyrinthitis ossificans can also result from temporal bone trauma, Leukemia, suppurative labyrinthitis, and autoimmune inner ear disease. Labyrinthitis ossificans can cause profound deafness and loss of vestibular function.


On the left images of a 56-year old male, who is a candidate for cochlear implantation. Small calcification in basal turn of cochlea as a result of labyrinthitis ossificans (arrows).

The treatment for this uses several different medications along with steroids as well as surgery and hearing aids. Since the occurrence of ossification virtually guarantees that hearing will not be restored the cochlear implant is a very important treatment option.

Wilson's Disease

Hyperintensities in the bilateral basal ganglia and thalami shown by T2-weighted MRI of the brain


Wilson's disease is a rare inherited disorder that causes the body to absorb and retain excessive amounts of copper. The accumulation of copper can damage many organs and tissues. The copper usually deposits in the liver, brain, kidneys, and the eyes. The liver and central nervous system are most often affected and are the most dangerous. If left untreated the copper can accumulate to life-threatening levels and can be fatal. Several different tests are used when diagnosing Wilson's disease. CT and MRI scans are used when diagnosing Wilson's of the brain. Other tests are also done to measure the amount of copper in the blood, urine, and liver. Although the copper accumulation begins at birth, symptoms of the disorder appear later in life. The most characteristic symptom of Wilson's is the Kayser-Fleisher ring which is a rusty brown ring around the cornea of the eye. Up to one-third of people with WD have neurological signs and symptoms, such as tremors, seizures, drooling, difficulty speaking, loss of fine motor skills, repetitive movements, slowness in movements. WD also causes behavioral and psychological problems such as abrupt personality changes, depression and neurotic behaviors.

If WD is detected early a person can enjoy a normal life and live a normal lifespan. Wilson's disease does require lifelong treatment by taking copper-reducing medication and avoiding copper containing vitamin and mineral supplements, along with foods high in copper. Wilson's disease itself is rare, but as many as one in 100 people has one defective ATP7B gene. WD is inherited and is an autosomal recessive trait, which means that to develop the disease you must inherit two copies of the defective gene, one from each parent. Exports recommend that all children and siblings of people with Wilson's be tested for disease.

This disease is very interesting to me because my ex-husbands family has a long history of Wilson's some cases even fatal and my daughter and grandchildren have been tested for this disease and are still being tested periodically for symptoms.