Chiarians Unite

Paroxysmal Rage

On occasion, I will post on some of the rare or uncommon presentations of the Chiari I malformation. In 1999, Rodger J. Hudgins from Scottish Rite Children’s Medical Center in Atlanta, Georgia, published an article in the Journal of Neurosurgery on a rare presentation in children. He described two children whose primary presenting feature was sudden attacks of rage.

The first case:

1.            “This 2.5-year-old boy presented with a several-month history or rage episodes in which he would suddenly and without warning become extremely irritable, upsetting furniture, biting and kicking those around him including his parents..... These spells would last for seconds to minutes and then end as rapidly as they began.”

Neurological examination was unremarkable except for “unsustained clonus and mild lower extremity hypertonicity. ” MRI revealed a Chiari I malformation with 20 mm of tonsillar herniation. The child was treated with a “suboccipital craniectomy and C1-2 laminectomy without duraplasty.” Following surgery, the rage episodes completely resolved and at one-year follow-up was noted to be developing normally.

The second case:

1.            “This 8-year-old girl presented with a 6-month history of episodic violent behavior in which she would run wild, scream, and be uncontrollable. During these outburst she had kicked and punched people around her. These episodes were sudden in onset, but could last most of the day and would occasionally end only when the child was exhausted.”

She was admitted for psychiatric evaluation. An MRI revealed a Chiari I malformation with 18 mm of tonsillar herniation. Neurological examination was normal. The rage episodes completely resolved after a “suboccipital craniectomy and C1-2 laminectomy without duraplasty.”  Eight months following surgery she was described as “pleasant and engaging.”

Lesson:

Irritation of the meninges in the foramen magnum and upper cervical canal is the presumed mechanism for the occipital pain of CM-I. Distress caused by pain may lead to raised intracranial pressure further increasing the headache. These two children expressed their pain as rage. Children with similar presentations should have an MRI of the brain with special attention to the posterior fossa.

Posted by John Oro’, MD

Posted Oct 18 08 12:43pm

http://stanford.wellsphere.com/chiari-malformation-article/paroxysmal-rage/428036

 

 

More on Rage

The post on Paroxysmal Rage has generated a comment from BJR directing our attention to a study by Maryniak & Roszkowski in children with cerebellar tumors and makes an important suggestion that the role of the cerebellum in emotional regulation, not pain, may have been responsible for the rage episodes in the two children with Chiari I malformation described by Rodger Hudgins.

The Maryniak & Roszkowski study supports the role of the cerebellum in disinhibition, impulsivity and irritability. Recent findings show that the cerebellum is involved in more than coordination (See post: The New Cerebellum ).

What about the paroxysmal nature of the spells in the two children studied by Hudgins? Are these paroxysms an expression of pain due to meningeal irritation? (As the agitation increases, the pain would further worsen and build to a paroxysm.) Or, are these paroxysms due to dysfunction of the cerebellum?

If you read Polish and can review the cerebellar tumor study by Maryniak & Roszkowski, please send your review to chiaritimes@mac.com. The review will be added to the site. Or, simply post your review in the Comments section.

Questions of particular interest:

1.  What were the average age, age range, and gender of the children?

2.  The symptoms of disinhibition, impulsivity and irritability were reported following surgery. Did the children have similar symptoms prior to surgery?

3.  How did disinhibition, impulsivity and irritability present itself?

4.  Where the emotional symptoms in these children paroxysmal, or of a more generalized nature?

5.  Did the symptoms improve with time and what was the length of follow-up?

The Maryniak & Roszkowski abstract can be found at this link in PubMed. A link to the full-text article is located on the right side of the PubMed page. Free registration is required.

More studies needed:

As we consider the origin of emotional symptoms in persons with cerebellar lesions or the Chiari I malformation, it is important to keep in mind the thoughts expressed by Schoch et al.:

1.            “Although a role of the cerebellum in specific aspects of non-motor functions seems obvious it is still an open question which cognitive functions are involved, why and to what extent. It is a matter of ongoing discussion whether or not cognitive dysfunction belongs to the symptoms of cerebellar disease. Overall, disorders appear to be mild and far less frequent than disorders observed following lesions of cerebral areas.”

Posted by John Oro’, MD

Posted Oct 18 08 12:42pm

Original posting on Wellspere:  http://stanford.wellsphere.com/chiari-malformation-article/more-on-rage/428035