Symptoms that develop and the progression of the disease will vary from patient to patient but usually will include:

  • Balance and coordination problems
  • Weakness
  • Poor concentration and apathy
  • Headaches or dizziness
  • Poor concentration
  • Abnormal movements
  • Challenging behaviour and sometimes aggression
  • Extreme anxiety and agitation
  • Confusion and memory lapse leading to rapidly progressive dementia
  • Visual disturbance often progressing to Cortical blindness
  • Deafness
  • Difficulty with speech leading to inability to communicate
  • Increased mobility problems
  • Marked startle reflex
  • Incontinence and/or retention of urine
  • Constipation
  • Difficulty swallowing and excess salivation
  • Abnormal posturing
  • Seizures
  • Inability to recognise people
  • Coma and death

Symptoms of Sporadic CJD

Early symptoms of sporadic CJD can include depression, mood swings, memory lapses, poor concentration, social withdrawal, lack of interest and uncharacteristic behaviour.

Patients often suffer impaired judgement confusion, or disorientation, which can rapidly advance to a dementia. They often also suffer perceptual problems which may lead to the patient misinterpreting what or who they see.

The patient will become unsteady on their feet, become clumsy and lack coordination (cerebellar ataxia) and may complain of blurred or double vision.

Other symptoms may include hallucinations, blindness, rigidity in the limbs, sudden jerking movements (myoclonus) and incontinence.

Patients usually become hypersensitive to sound, touch, and/or light.

The patient will become bed bound, speech can become slurred and swallowing difficult (dysphasia). Eventually, the patient loses the ability to move and speak and they will require full time nursing care. A state clinically known as akinetic mutism usually develops, with the patient not be able to communicate with others or perform gross motor tasks such as moving their limbs voluntarily. The rapid decline, particularly with sporadic CJD, is very common.

Symptoms of Genetic CJD

Symptoms can vary depending on the type of PRNP mutation involved. There can be a great variation in the symptoms affecting patients even within the same family. In many cases the symptoms are the same as for sporadic CJD and it is only with a gene test that a genetic cause can be established or ruled out.

Gerstmann Sträussler Scheinker Disease (GSS)

GSS usually starts with cerebellar ataxia and progresses to dementia. The patient can survive for several years.

Fatal Familial Insomnia (FFI)

In FFI, the main symptom is typically a progressive and untreatable form of insomnia and autonomic dysfunction (e.g. labile blood pressure and temperature). The damage to the brain is mostly confined to the thalamus, the area which plays a role in controlling sleep-wake cycles.

Symptoms of Variant CJD

Variant CJD is clinically quite different to classical CJD.

The early symptoms of vCJD are generally psychiatric. These include depression, anxiety, apathy, withdrawal and delusions. Most vCJD patients are usually considered to be suffering from purely psychiatric disorders during the early phase of the illness. The symptoms are persistent and patients are usually prescribed psychiatric medications and seen by psychiatrists. Definite neurological symptoms do not generally develop until after approximately six months, with memory impairment, persistent sensory symptoms (such as tingling, burning or pain), ataxia (balance and walking disturbances), involuntary movements and visual disturbances all frequently seen. The illness progresses to include a dementia and a state of complete helplessness. Death on average occurs after 14 months (with a range of eight to thirty-eight months) from disease onset and at an average age of 28 years (ages of those dying from vCJD have ranged from 14 to 74 years of age).

BSE has NOT been found in Australian livestock and to date there have been no reported cases of vCJD in Australia.