Benson syndrome is a rare type of vision problem (2023)

Posterior cortical atrophy, also called Benson syndrome, is a neurodegenerative disorder of the brain that produces a range of visual symptoms. Loss of visual abilities is the predominant effect of this condition. If you or a loved one is diagnosed with posterior cortical atrophy, the effects can be frustrating.

Because the visual changes in Benson syndrome are complicated, it may take some time for your medical team to arrive at the correct diagnosis. There is no effective cure for posterior cortical atrophy, and supportive care and coping strategies are the mainstay of treatment for this condition.

Symptoms

Posterior cortical atrophy affects adults over age 50 and usually begins before age 65. Once symptoms begin, the condition slowly progresses. Visual changes are the most prominent features of posterior cortical atrophy. Depression, anxiety, dementia and loss of certain cognitive skills can also develop, especially in the later stages of the disease.

It can be difficult to recognize the symptoms of posterior cortical atrophy because they are related to visual abilities rather than pure visual deficits. These alterations are described as loss of visuoperceptive and visuospatial abilities and not as blindness or vision loss.

The effects of posterior cortical atrophy include:

• Difficulty reading or writing
• Impaired recognition of objects or people.
• Problems with the perception of distances.
• Decreased object recognition.
• Inability to identify moving objects or discern that objects are moving
• Confusion when looking at various objects.
• visual hallucinations
• Depression
• A feeling of powerlessness
• Anxiety
• trouble remembering words
• problems with calculations

Posterior cortical atrophy is often considered a variant of Alzheimer's disease, although memory loss usually does not develop until years after the onset of the disease.

If you or a loved one develops posterior cortical atrophy, the effects can be particularly disabling if they also have other neurological conditions, such as a stroke or Parkinson's disease.

Causes

Posterior cortical atrophy is caused by neurodegeneration, which is the loss of neurons. In this condition, neurons in the posterior region of the brain deteriorate over the years, resulting in atrophy (shrinking) of the posterior cerebral cortex.

The posterior cerebral cortex of the brain includes the left and right occipital lobes, which are adjacent to each other. The occipital lobes mediate visual perception, allowing people to understand and recognize what their eyes see.

There is no known cause or trigger for posterior cortical atrophy, and there is no particular gene or inheritance pattern associated with the condition.

variant of Alzheimer's disease

Posterior cortical atrophy has been considered a type ofAlzheimer's disease. There are some similarities and differences between posterior cortical atrophy and Alzheimer's disease.

Both conditions have similar characteristics in post mortem (after death) research studies. In posterior cortical atrophy and Alzheimer's disease, the brain contains amyloid plaques and neurofibrillary tangles. These are proteins that are produced in the process of neurodegeneration.

An important difference between the conditions is that Alzheimer's disease is generally characterized by atrophy of the medial portions of the temporal lobes, resulting in difficulties with short-term memory early in the course of the disease. On the other hand, the loss of visual abilities, characteristic of posterior cortical atrophy, is not typical of Alzheimer's disease.

Diagnosis

Posterior cortical atrophy is diagnosed based on clinical symptoms, physical examination, and diagnostic tests. If you or a loved one develops this condition, you cannot specifically complain about the visual changes. Instead, he may complain about changes in behavior or confusion.

Physical exam

Your physical exam includes a neurological exam, which tests muscle strength, reflexes, sensation, coordination, gait, vision, speech, and memory. These may be normal in posterior cortical atrophy, but visuospatial difficulties may make it difficult to cooperate with the exam. .

vision tests

Further evaluation may include visual acuity tests and an eye examination, which are usually normal. Visual acuity tests measure your ability to see objects clearly in the distance, usually using a reading chart. This test does not involve recognizing complex relationships between objects or knowing the names of objects or what they are used for.

neuropsychological tests

You may need tests that specifically test your memory, concentration, problem-solving skills, and judgment. These tests are often interactive, involve your participation, and can take hours. Neuropsychological tests can be helpful when trying to pinpoint your exact neurological deficits.

imaging exams

There is a high chance that you will have a brain imaging test such ascomputed tomography (CT)onuclear magnetic resonance (NMR). These tests can identify injuries to the brain, such as strokes, traumatic brain injuries, and areas of atrophy.

In posterior cortical atrophy, the occipital lobes are smaller than would normally be expected, and this is usually evident on CT and MRI scans of the brain.

blood test

You may also have some blood tests, including acomplete blood count (CBC)and electrolyte tests. These tests can help determine if you have another problem, such as an infection or metabolic problem that could be causing your symptoms.

Lumbar puncture

A lumbar puncture (PL), also called a lumbar puncture, is an invasive diagnostic test. If you have this test, your doctor will place a needle in your lower back to collect spinal fluid. The procedure takes five to 10 minutes and is a little uncomfortable, but it is safe and easily tolerated by most people.

An LP can identify evidence of an infection or inflammation (as in multiple sclerosis). Results would normally be normal in posterior cortical atrophy.

Differential diagnosis

There are several medical conditions that can cause similar effects to posterior cortical atrophy, and it can be difficult to tell the difference in the early stages of the disease.

Blindness/vision loss: Decreased vision may manifest with symptoms similar to those of posterior cortical atrophy. Vision tests can distinguish true vision loss from posterior cortical atrophy.

Schizophrenia: Psychosis is often associated with hallucinations, which are false visual or auditory stimuli. Schizophrenia often causes behavioral changes and hallucinations and may mimic subsequent cortical atrophy. A careful medical history and physical exam can tell the difference between these conditions. Schizophrenia is not associated with brain atrophy.

Insanity: Various types of dementia, includingdementia with Lewy bodies,pick's disease,vascular dementia, and Alzheimer's disease, can cause behavioral changes.

Depending on the pattern of neurological deficits, these types of dementia can be confused with each other or with subsequent cortical atrophy early in the diagnostic process. They often have different characteristics, and as you progress, the difference between them becomes clearer.

However, as each of these types of dementia continues to worsen, they can become so severe that some of their late-stage effects can be almost indistinguishable.

Attack: AattackIt is a sudden brain injury that occurs due to insufficient blood supply to the brain. A stroke can cause a variety of different effects, depending on the location of the impaired blood supply.

An occipital stroke can cause symptoms similar to posterior cortical atrophy, but the symptoms come on suddenly and do not progress. In addition, imaging tests can often identify strokes.

Infection: Any infection in the brain, such asmeningitis(an infection of the protective meningeal covering of the brain) or encephalitis (an infection of the brain), can cause behavioral symptoms. These infections usually cause fever and abnormal LP.

encephalopathy: Systemic conditions that affect the body can affect brain function and produce a variety of symptoms, including visual changes. Wernicke's encephalopathy, liver failure, kidney disease and cancer are examples of diseases that can cause visual changes.

These conditions are more common than posterior cortical atrophy and can be misdiagnosed early in the disease. The blood test is usually normal in posterior cortical atrophy and abnormal in metabolic encephalopathy.

Inflammation: Inflammatory conditions such aslupusmay have neurological effects that may mimic posterior cortical atrophy. However, in most inflammatory disorders, other systemic symptoms are present to help confirm the diagnosis.

Treatment

There is no single definitive treatment for posterior cortical atrophy. However, there are some steps you can take to optimize your skills and quality of life.

Therapy

Specialist rehabilitation and vision therapy can be helpful, especially if you don't have symptoms of dementia. It is important to be patient and persistent when it comes to therapy and to try to work with a therapist who is familiar with visuospatial impairment treatment.

Assistance and Care

You may need help with your daily activities from a professional caregiver or family members. As with rehabilitation therapy, it is important for caregivers to become familiar with the effects of posterior cortical atrophy and learn to be alert to potentially harmful situations.

A Word from Verywell

If you or a loved one has been diagnosed with posterior cortical atrophy, there's no doubt that it can be challenging. Security can also be an issue, and it is vital that you and your family pay attention to making your home and surroundings as safe as possible. how is it possible.

Not only is the condition itself difficult to live with, but the fact that it is so rare also makes it difficult to find guidance and support. It's important that you don't hesitate to ask for help when you need it. Your medical team can help prescribe the appropriate therapy for your condition.