| 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 | |
| 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 rareseizure disorder in dogs, characterized by explosive aggression.[1][2][3]
It is frequently confused withidiopathic 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.[4] It is treated withantiepileptics.[5]
Rage syndrome has been known by a variety of names since it was discovered.[1][3] 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.[1]
Rage syndrome is also sometimes termed a form of epilepsy, particularly limbic epilepsy.[6][7] The term limbic epilepsy for rage syndrome has been used synonymously with the terms psychomotor epilepsy, automatic epilepsy, rhinencephalic epilepsy, behavioral epilepsy, temporal lobe epilepsy, and autonomic epilepsy.[6][7][8][9][10] In addition, depending on the professional's discretion the word "seizure" may be substituted for "epilepsy" in a given study or resource.[7] Edward B. Breitschwerdt, a researcher and veterinarian, wrote that this "profusion of synonyms makes it difficult to clearly ascertain the results of either clinical or research investigations of limbic epilepsy in the dog".[6]
Rage syndrome is frequently confused withidiopathic 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 was sometimes referred to as sudden-onset idiopathic aggression (SOIA) to distinguish it fromidiopathic 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 nounrabiēs, meaning "rage").
Aggression in rage syndrome is characterized by its severity and oftenfatal to dogs, animals, or people it is targeted towards.[1] Episodes may cause life-threatening injuries and result indisability ordisfigurement.[1]
Dogs with rage syndrome typically have their first rage episode during adolescence, between 1 and 3 years old, similar to dogs withidiopathic epilepsy.[1]
InterictalEEGs undergeneral anesthesia typically show low-voltage rapid discharges characteristic offocal seizures.[1][11][12] Seizure foci are in thetemporal lobe, most often theleft temporal lobe.[1][13]
Prognosis for treatment of rage syndrome is guarded.[1] It is treated withantiepileptics.[5]
TheEnglish Springer Spaniel is the origin of the term "Springer Rage" frequently used in the 1970s and 1980s.
English Springer Spaniels were the breed most often referred todog behavior consultants for aggression in the 1980s.[2][14][15]
Pat Miller wrote inBeware 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."[16]
Bull terriers have also been used in research studies on breed-specific hereditary focal seizures, sometimes includingaggressive symptoms.[17]
Rage syndrome is diagnosed by EEG.[1][4][13]
Thyroid function istested during typical diagnostic workups as thyroid conditions, most commonlyhypo- andhyperthyroidism, have been suggested to cause pathophysiological aggression that may present similarly to Rage syndrome.[1][18]
In 2002 a 4-month-old tigress was reported with symptoms analogous to rage syndrome.[13] The tigress had episodes between 30 seconds and a minute long of explosive self-directed aggression and self-mutilation, in addition to occasionalgeneralized tonic-clonic seizures and bothfocal and generalized neurologic symptoms such as episodes of continuously walking in circles to the right andepileptic 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 fromfaintly elevated CSF proteins. The tigress was the result of aconsanguineous breeding and one of her littermates was diagnosed with generalized seizures. She was successfully treated withphenobarbital (2.5 mg/kg by mouth twice a day, bringing her within the therapeutic range at a blood concentration of 24 mg/dl).[13]
In 1980 it was suggested thatelectroconvulsive therapy,prefrontal lobotomy, andpartial cerebral hypoxia (hypoxic-anoxic brain injury) be used to treat rage syndrome.[1] A 1974 study on prefrontal lobotomy of aggressive dogs found that 50% of lobotomized dogs died of complications.[19] Surviving dogs developedseizures,fecal andurinary incontinence,confusion,memory loss,seromas,emotional dysregulation, focal neurologic symptoms,weakness, andweight loss.[19] 40% of surviving dogs did not have a reduction in existing aggression, and some dogs' aggression worsened after the procedure.[19] The treatments were described asheroic measures which caused further damage to the dogs' health but were undertaken as a last resort to preserve the lives of the dogs. Multiple dogs which did not die from the procedure itself were euthanized anyway.[19]
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