Rage syndrome

Not to be confused with Idiopathic aggression.

Rage syndrome
Other names	A22, episodic dyscontrol, stimulus-responsive psychomotor epilepsy, Mental lapse aggression syndrome
The Malinois was the first breed to have a specific genetic polymorphism linked to rage syndrome.
Specialty	Neurology
Symptoms	Lethal dog attacks Dog bites Seizures
Complications	Behavioral euthanasia
Usual onset	1–3 years old
Duration	Lifelong
Causes	Unknown
Risk factors	PolyA(22) (in Malinois), DAT-VNTR (in Malinois), likely other genetic factors, breed, other seizures
Diagnostic method	EEG, with complete exclusion of other medical conditions via thorough medical testing
Differential diagnosis	Any untreated illness (Pathophysiological aggression) or behavioral condition
Prevention	Selective breeding, Health testing (PolyA(22) test, in Malinois)
Management	Anticonvulsants, muzzling, barrier use, lifestyle changes
Medication	Antiepileptic drugs (Phenobarbital)
Prognosis	Generally poor

Rage syndrome is a rare seizure disorder in dogs characterized by explosive aggression.

It is frequently confused with Idiopathic aggression, a term for aggression with no identifiable cause. Rage syndrome is most often a misdiagnosis of dogs with an unrelated, but more common, form of aggression. Although the scientific evidence is limited, it is thought to be genetic in origin, and is heritable. It is treated with antiepileptics.

Names

Rage syndrome has been known by a variety of names since it was discovered. Rage syndrome is a colloquial term most often preferred by dog trainers, handlers, and some behavior consultants. Alternative names used by researchers, veterinary scientists, and behavior specialists include mental lapse syndrome (MLS) and episodic dyscontrol.

Rage syndrome is frequently confused with Idiopathic aggression, a term for aggression with no identifiable cause, due to sharing a name with it in its earliest studies before being identified as a discrete condition. In early research Rage syndrome is sometimes referred to as sudden-onset idiopathic aggression (SOIA) to distinguish it from idiopathic disease, and later as sudden-onset aggression (SOA).

Rage syndrome has no medical connection to rabies, for which its name is sometimes mistaken (from the Latin noun "rabiēs", meaning "rage").

Characteristics

Aggression in rage syndrome is characterized by its severity and often fatal to dogs, animals, or people it is targeted towards. Episodes may cause life-threatening injuries and result in disability or disfigurement.

Dogs with rage syndrome typically have their first rage episode during adolescence, between 1 and 3 years old, similar to dogs with idiopathic epilepsy.

Interictal EEGs under general anesthesia typically show low-voltage rapid discharges characteristic of focal seizures. Seizure foci are in the temporal lobe, most often the left temporal lobe.

Risk factors

The English Cocker Spaniel, Belgian Malinois, and English Springer Spaniel are the breeds most commonly associated with Rage syndrome.

Associated breeds

English Springer Spaniel

The English Springer Spaniel is the origin of the term "Springer Rage" frequently used in the 70s and 80s.

English Springer Spaniels are the breed most often referred to dog behavior consultants for aggression.

Pat Miller wrote in Beware of the Dog: Positive Solutions for Aggressive Behavior in Dogs in 2017: "[Rage syndrome] captured the imagination of the dog world, and soon every dog with episodes of sudden, explosive aggression was tagged with the unfortunate "rage syndrome" label, especially if it was a Spaniel of any type."

Diagnosis

Rage syndrome is diagnosed by EEG.

Differential diagnosis

Endocrine

Thyroid function is tested during typical diagnostic workups as thyroid conditions, most commonly hypo- and hyperthyroidism, have been suggested to cause pathophysiological aggression that may present similarly to Rage syndrome.

In other animals

In 2002 a 4-month-old tigress was reported with symptoms analogous to Rage syndrome. The tigress had episodes between 30 seconds and a minute long of explosive self-directed aggression and self-mutilation, in addition to occasional generalized tonic-clonic seizures and both focal and generalized neurologic symptoms such as episodes of continuously walking in circles to the right and epileptic blindness. Her EEG showed left frontal-temporal epileptiform activity, confirming a diagnosis of complex partial seizures. Cerebrospinal fluid testing, hematologic testing, serotologic testing, and serum biochemistry profile were all normal aside from faintly elevated CSF proteins. The tigress was the result of a consanguineous breeding and one of her littermates was diagnosed with generalized seizures. She was successfully treated with Phenobarbital (2.5 mg/kg by mouth twice a day, bringing her within the therapeutic range at a blood concentration of 24 mg/dl).

Superseded theories

In 1980 it was suggested that electroconvulsive therapy, prefrontal lobotomy, and partial cerebral hypoxia (hypoxic-anoxic brain injury) be used to treat rage syndrome. A 1974 study on prefrontal lobotomy of aggressive dogs found that 50% of lobotomized dogs died of complications. Surviving dogs developed seizures, incontinence, confusion, memory loss, seromas, emotional dysregulation, focal neurologic symptoms, weakness, and weight loss. 40% of surviving dogs did not have a reduction in existing aggression, and some dogs' aggression worsened after the procedure.

See also

• Phenobarbital
• List of fatal dog attacks
• Causes of seizures
• Animal euthanasia

Further reading

• Lit, Lisa; Belanger, Janelle M; Boehm, Debby; Lybarger, Nathan; Haverbeke, Anouck; Diederich, Claire; Oberbauer, Anita M (30 May 2013). "Characterization of a dopamine transporter polymorphism and behavior in Belgian Malinois". BMC Genetics. 14 (1). Springer Science and Business Media LLC: 45. doi:10.1186/1471-2156-14-45. ISSN 1471-2156.{{cite journal}}: CS1 maint: unflagged free DOI (link)
• Lit, L.; Belanger, J. M.; Boehm, D.; Lybarger, N.; Oberbauer, A. M. (9 April 2013). "Dopamine transporter single nucleotide polymorphisms (SNPs) in Belgian Malinois dogs". Animal Genetics. 44 (5). Wiley: 604–605. doi:10.1111/age.12040. ISSN 0268-9146.
• Dodman; Miczek, K. A.; Knowles, K.; Thalhammer, J. G.; Shuster, L. (1992). "Phenobarbital-responsive episodic dyscontrol (rage) in dogs". Journal of the American Veterinary Medical Association. 201 (10): 1580–1583. PMID 1289339. Retrieved 22 May 2023.
• B.V., Beaver (1980). "Mental lapse aggression syndrome [Dogs]". Journal American Animal Hospital Association: 937-939. ISSN 0587-2871. Retrieved 22 May 2023.