Progressive supranuclear palsy

Clinic

• PSP is a late-onset degenerative disease involveing
  • Basal ganglia: Particularly the subthalamic nucleus, substantia nigra, and globus pallidus
  • Brain-stem: Particularly Tectum (the portion of the midbrain where "supranuclear" eye movement resides), as well as dopaminergic nuclei.
  • Cerebral cortex: Particularly Frontal lobes and Limbic system (Similar to frontotemporal degeneration)
  • Dentate nucleus of the cerebellum
  • Spinal cord: Particularly the area where some control of the bladder and bowel resides

Nero-Degenerative disease

• It is an umbrella that contain
• PSP
• Corticobasal degeneration
• Frontotemporal dementia
• Pick's disease.
• Parkinson disease
• Alzheimer disease

Signs / Symptoms

The initial symptoms in two-thirds of cases are

• Loss of balance: Lunging forward when mobilizing, fast walking, bumping into objects or people, and falls
• Dementia is initially seen in 20 %of frontotemporal dementia
• Slowing of movement
• Difficulty moving the eyes
• Cognitive impairment

Other common early symptoms are

• Changes in personality
• General slowing of movement
• Visual symptoms
• The most common behavioural symptoms include
  • Apathy
  • Lack of inhibition
  • Anxiety
  • Profound state of unease or dissatisfaction
• Later symptoms and signs can include, but do not necessarily include
• Dementia (typically including loss of inhibition and ability to organize information)
• Slurring of speech
• Difficulty swallowing
• Difficulty moving the eyes, particularly in the vertical direction.
• Some of the other signs are
  • Poor eyelid function
  • Contracture of the facial muscles
  • Backward tilt of the head with stiffening of the neck muscles,
  • Sleep disruption
  • Urinary incontinence
  • Constipation
  • Some patients retain full cognitive function up to the end
  • The visual symptoms are of particular importance in the diagnosis of this disorder. Patients typically complain of difficulty reading due to the inability to look down well. The ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement and the inability to make vertical saccades, which is often worse with downward saccades. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. This vertical gaze paresis will correct when the examiner passively rolls the patient's head up and down as part of a test for the oculocephalic reflex. Involuntary eye movement, as elicited by Bell's phenomenon, for instance, may be closer to normal. On close inspection, eye movements called "square-wave jerks" may be visible when the patient fixes at distance. These are fine movements, that can be mistaken for nystagmus, except that they are saccadic in nature, with no smooth phase. Although healthy individuals also make square-wave jerk movements, PSP patients make slower square-wave jerk movements, with smaller vertical components. Assessment of these square-wave jerks and diminished vertical saccades is especially useful for diagnosing progressive supranuclear palsy, because these movements set PSP patients apart from other parkinsonian patients. Difficulties with convergence (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia (double vision) when reading.

A characteristic facial appearance known as “procerus sign”, with a wide-eye stare, furrowing of forehead with a frowning expression and deepening of other facial creases is diagnostic of PSP.

Note

In order to have a good miasmatic analysis, we d better to have some eliminating Sigh / Symptoms. I choose initial symptoms Dementia, Cognitive impairments, Ataxia.

Therefore NVCJD and TBE are omitted.

Entities / Miasms

		GSS	FFI	NVCJD	TBE
	Ataxia	+++	+++		+++
	Dementia	+++	+++	+++
	Cognitive impairment	+++	+++	+++	+++
	Dysarthria	+++	+++	+++
	Depression	+++	+++	+++	+++
	Emotional lability	+++			+++
	Dysphagia		+++	+++	+++
	Urinary incontinence				+++
	Sleep disruption		+++	+++
	Face palsy				+++
	Constipation				+++