​Primary Progressive Aphasia

Primary progressive aphasia is a rare neurological disease, that is, a disease relating to the brain. It affects your language capabilities, which become slowly and progressively impaired. However, your other mental functions are not impacted. While other forms of aphasia result from stroke or traumatic brain injury, primary progressive aphasia is a degenerative brain condition. It results from worsening of brain tissue affecting areas of the brain that are important for speech and language.

Primary progressive aphasia usually begins as a subtle language disorder, but progresses to almost a complete inability to speak in severe cases. The type or pattern of the language deficit may differ from patient to patient. Initially, you may have a normal or even increased capacity of word production. However, over time your speech becomes less fluent and slower and requires more effort. In some cases it begins with a reduced ability to find the right words but progressively becomes worse, affecting your overall articulation. ​​

The main symptoms of primary progressive aphasia are:

  • ​Slowed or halting speech

  • Decreased use of language

  • Hesitation in finding the right words

  • Abnormal order of words while speaking or writing

  • Incorrect substitution of words 

  • Using words that are mispronounced or incomprehensible

  • Difficulty understanding or following normal conversations

  • Decreased ability to understand simple words

  • Forgetting the names of familiar objects

  • Inability to recall people’s names 

  • Problems in writing 

  • Problems in reading 

  • Unusual spelling mistakes

  • Problems in arithmetic and calculations ​

Adults of any age can develop primary progressive aphasia, but it is more common in people under the age of sixty five. People with primary progressive aphasia can have a variety of different language symptoms and no two cases are exactly the same.​

If you or a loved one is experiencing language problems or any of the symptoms mentioned above, it is recommended that you consult with your doctor immediately. You can obtain additional information and expert medical advice from the highly trained and internationally accredited staff at the <Mind and Brain Service Line> at The Aga Khan University Hospital.​

Your time with your doctor may be limited, so make sure to prepare for your visit beforehand. Here are some tips to help get you started.​

Your doctor will first take a careful and detailed family and medical history. Family members or friends may also be questioned about your behaviour because sometimes you may be unaware of the symptoms yourself. A neurological examination will be done to determine your mental status as well as check for signs of deterioration of motor or sensory skills. Several blood tests, spinal fluid tests and brain imaging studies (such as MRI - Magnetic Resonance Imaging or a CAT - Computerized Axial Tomography scan) can be conducted. Computerized images of your brain can be used to check for abnormalities such as atrophy (shrinkage) of the brain tissue. However, in early stages, these may not show up on the scan. 

Neuropsychological examination will be the next phase of testing. This involves evaluating whether your symptoms are psychiatric. These are detailed tests and require several hours of testing. Tests may be paper or computer based and their basic function is to test your mental abilities such as attention span, concentration, language, learning/memory, visual perception, reasoning and mood. The results of these tests can indicate if there are abnormalities of thinking and behaviour, and whether these are mild, moderate or advanced. 

Speech and language evaluation is a crucial test as a decline in language abilities is the primary symptom of primary progressive aphasia. Your doctor will attempt to determine which components of language are most affected, their severity, and the measures that can be taken to improve communication. 

Family members will be included in the treatment sessions to educate them about how to facilitate communication.​

At present, there is no cure for primary progressive aphasia. However, doctors and other health care professionals can help you manage your condition. Medical specialists working with the <Mind and Brain Service Line> at The Aga Khan University Hospital are equipped to provide comprehensive, state-of-the-art medical care, and discuss with you the measures being undertaken. 

There are no drugs that specifically treat this disease. Speech and language therapy has been a useful option in some cases. By working with a speech and language pathologist, you can make an effort to compensate for your loss of language skills. It cannot prevent or slow the progression of your disease, but it can help you to manage your condition better and be able to communicate in a better way.​

The Aga Khan University Hospital offers various support services to help with managing or recovering from the disease or condition. These include but are not limited to nutrition, physiotherapy, rehabilitation, specialized clinics and some patient support groups. Your doctor or nurse will advise you accordingly.

The Aga Khan University Hospital offers financial assistance to those who are in need and fulfil the eligibility criteria. For further information, you can contact the Patient Welfare Department. You can find the contact number of the Patient Welfare Department in the ‘Important Numbers’ section on the website homepage.​

The financial counselling staff is available during office hours, at the main PBSD (Patient Business Services Department), to answer your financial queries on treatments’ costs and authorize admissions on partial deposit as per hospital policies allow. The financial counsellor in the emergency room is open 24/7. You can find the contact number of the Patient Business Services in the ‘Important Numbers’ section on the website homepage.​

Your doctor and or nurse will give you specific instructions about the prescribed medication. Please ensure that you take or use the prescribed medicine as advised. It can be dangerous to your health if you self-prescribe. Please inform the doctor or nurse beforehand if you have experienced any adverse reactions to any medications in the past. If you experience any symptoms of drug poisoning, overdose or severe reaction please contact the Pharmacy Service at The Aga Khan University Hospital immediately. You can find the contact number of the Pharmacy Services in the ‘Important Numbers’ section on the website homepage.​

The information provided on our website is for educational purposes and not intended to be a substitute for medical advice, diagnosis or treatment. You should always seek the advice of your doctor or other healthcare professional provider.