Symptoms

Not all patients present the same.

HE/SREAT patients typically present with serious, debilitating symptoms that usually include several – but not all – of the following:

  • confusion and disorientation
  • concentration and memory problems
  • personality changes
  • speech difficulties (usually transient)
  • tremors
  • muscle jerking (myoclonus)
  • impaired coordination and balance (ataxia)
  • headaches
  • disorientation
  • seizures or seizure-like events
  • partial paralysis  (transient)
  • sleep abnormalities
  • psychosis
  • coma

The condition tends to follow a relapsing and remitting course, although long-term remission has been reported in some cases. Left untreated, HE/SREAT can – in some cases – lead to coma or even death, particularly in pediatric patients where status epilepticus is not uncommon.

HE/SREAT patients are predominantly – but not exclusively – female.  Although the average age of onset is between 45 and 60, in recent years more and more cases of pediatric HE/SREAT have been reported.

There are two types of clinical presentation: The first and most common type is relapsing and remitting, and it is characterized by acute stroke-like episodes with transient focal neurological deficits.  The NIH refers to this first type as “vasculitic.”

The second, less common presentation of HE/SREAT is more insidious in onset, and tends to be progressive. It can result in dementia, and psychosis.

Many patients display an overlap in symptoms between the two types.

 

Last edited by Web Team on June 20th, 2016