Confusional Migraine

General

Confusional Migraine has come to refer to the development of acute altered sensorium with confusion, irritability, drowsiness, and nausea or vomiting following mild non-concussive head injury.  At times confusional migraine may occur spontaneously.  Most of the patients have throbbing headache when they recover enough to describe how they feel and have had or go on to develop migraine headache.  There is an increased history of migraine in the family of affected patients.  Tests such as CAT or MRI scan of the brain are normal although the EEG is typically slow during the attack and becomes normal after recovery.  The syndrome occurs mostly in young children or adolescents who remain amnestic for what happened after the event.  Occasional patients may have recurrent attacks.  Acute care physicians should have this syndrome in their differential diagnosis along with the other possible and more concerning neurologic diseases such as subdural, epidural, or subarachnoid hemorrhage, meningitis, nonconvulsive seizure, postictal state, or metabolic disorder.

Literature review

Liveing wrote in 1873:[i]

We have next to consider the disturbance of the higher cerebral faculties which sometimes attends the megrim paroxysm; this may be regarded for the most part as either intellectual or emotional.  The former is represented in some instances by loss or impairment of memory, and in others by confused, incoherent, or tumultuous ideation, very rarely by hallucination; the later by general depression, or vague subjective feelings of anxiety and dread.

At this period (i.e., following the affections of sight and touch) her intellect becomes confused, and her memory at the same time, for about half an hour, so much impaired that she cannot even remember the name of any medicine she has just taken…

… as the visual phenomenon passed off his memory usually fail so much that for a time he was mentally incapacitated and whatever he read or did during that period left no impression…”

Liveing wrote an encyclopedic monograph[ii] in which he recorded the mental symptoms that his patients had experienced during attacks.  Sixteen out of 67 of his patients had confusion, trouble with memory, or a disturbance of consciousness.  Nine of the 67 patients had the onset of migraine before age 20, and the youngest was at age 9.

After Liveing confusion as part of a migraine attack was rare in the neurologic literature.  Whitty in 1953[iii] writing about “Familial hemiplegic migraine” described a 52-year-old woman who had prolonged confusion for a week after a migraine attack.  Selby and Lance in1960[iv] wrote about mental confusion as a feature of their migraine in 14 of 396 patients.  Wolff[v] in 1963 reported a 20-year-old female with migraine who developed mental clouding with hemiplegia and aphasia.

Gascon and Barlow[vi] writing in 1970 in Pediatrics on "Juvenile Migraine, Presenting as an Acute Confusional State" said the following regarding childhood migraine:

Migraine in childhood differs from that an adult life in several ways.  Attacks are generally shorter, although occasionally a bout is prolonged and may last several days.  Prodromal symptoms, such as visual phenomena, are less marked, and gastrointestinal upset is more intense.  Often gastrointestinal stress is a sole manifestation, as in the syndrome of "cyclic vomiting of childhood," which we frequently find to represent a form of migraine ontogenetically preceding more recognizable attacks with headache.

Gascon and Barlow stated that the purpose of their paper was, "to call attention to a clinical form of the migraine syndrome where the most prominent manifestation of the attack was impairment of the sensorium resulting in an acute confusional state."  They summarized the clinical features of their 4 patients by stating:

Our patients all showed defects of sensorium, impaired awareness of their environment, and slow response to external stimuli such as pinprick or questions from examiners.  One boy, the youngest in the group at age 8, manifested hyperactivity, restlessness, and combativeness, accompanied by obscene language.  When memory was specifically testable, there was a defect in recent memory, with partial retrograde amnesia for the events preceding the attack.

Emery wrote in 1977[vii] in Pediatrics about "Acute Confusional State in Children With Migraine."  He pointed out that:

a wide variety of paroxysmal visual, sensory, and motor disturbances with or without headache is included in the migraine syndrome, but disturbances of consciousness and mental functioning as part of the migraine syndrome in children are not well recognized.

Emery presented 4 patients aged 8-13 and 2 of them had mild preceding trauma.  Emery’s Case 1 follows:

An 8-year-old girl fell off her sled and rolled down a hill.  After she walked home approximately 400 m, her parents observed confused, fearful, agitated, and combative behavior.  Because this abnormal behavior persisted in the emergency room of the local hospital, she was transferred to the Medical Center Hospital of Vermont.

Her first EEG performed 7 hours after onset of symptoms and when she was still confused and agitated, showed a high voltage polymorphic delta activity more dominant over the left side.  This child remained agitated and confused for approximately 9 hours when she fell asleep and after she awoke 4-5 hours later she was alert and had a normal neurologic exam.  She was followed for 3 years and had intermittent, severe headaches.

Regarding Case 2 Emery described:

A 5 year-old boy, playing outside with his brother, slipped on the ice and bumped his forehead, without loss of consciousness.  He came into the house, ate lunch, and complained of a headache.  After napping for one hour, he acted confused and did not recognize his mother.  His speech was garbled.  He was not blind.  When examined in the emergency room of the local hospital, he is described as appearing pale and sweaty, with slurred speech and "talking nonsense."  He vomited several times.  He had a past history of mild, infrequent, and nonspecific headaches.  His mother had a history of bifrontal, throbbing headaches associated with nausea.

When the child was examined at the Medical Center Hospital of Vermont approximately five hours after the onset of the confused state, he would give his name on request, but the remainder of his speech was either unintelligible or incoherent.  When re-examined one hour later he was coherent and oriented, but lethargic.  He was amnesic for the 5 hour period between his nap and admission to the hospital.

The boy in Case 2 had an EEG performed 12 hours after the onset of his confusional state which showed a bioccipital high-voltage delta wave more prominent over the right hemisphere.  A third EEG 3 weeks later was normal.  During his 4 day hospitalization his exam was normal although he complained of intermittent, unilateral, alternating headache and abdominal pain.

Menken wrote in 1978[viii] in Clinical Pediatrics on "Transitory Confusion After Minor Head Injury," in which he described two boys, ages 16 and 15 who had severe confusion after minor head trauma.  Menken thought that the:

antecedent head trauma seemed so trivial that it was initially ignored in the differential diagnosis which considered toxic and metabolic encephalopathies, encephalitis, ictal or postictal confusional states, and drug intoxication.

He also described a common problem in pediatric neurology:

Neurologists and neurosurgeons with pediatric experience are familiar with the not infrequent syndrome of irritability, vomiting, and variable somnolence following minor head trauma.  These children are difficult to examine, resisting funduscopy for example, and drift off to sleep when unattended.  The basis for the syndrome is unknown since it is fully reversible and no pathologic material has been studied.

Ehyai and Fenichel in 1978[ix] wrote in Child Neurology on "The Natural History of Acute Confusional Migraine.”  They described 4 cases in children aged 9 to 14 years old.  They felt that in these patients "typical migraine headaches always developed eventually."  They noted that when the patient was first seen in a confused state and there was no history of migraine it was difficult to make a diagnosis so that a family history of migraine became an important clue.  They reported on the “natural history” of the problem by following Case 1 for 21 months, Case 2 for 18 months, Case 3 for 5 years, and Case 4 for 26 months.

Ehyai and Fenichel’s Case 5 follows:

A 14-year-old girl suddenly became disoriented while at school.  She was agitated and walked purposely in the hall, complaining of blurred vision.  Her speech was rapid but incomprehensible.  The agitated confusion lasted for a few hours; then she fell asleep and awoke the next morning with amnesia for the event.  A second identical episode occurred two days later.

She was examined at our hospital one week later and found to be normal.  Both the patient and her mother had had typical migraine for several years.  The patient's most recent migraine occurred two weeks prior to the first confusional episode.  She was treated with sublingually administered ergotamine, which was of some benefit.  During 26 months of observation, no further confusional states were recorded.

Ferrera and Reicho[x] writing in the American Journal of Emergency Medicine in 1996 on “Acute confusional migraine and trauma-triggered migraine” described 2 cases of children with histories of “confusion and agitation.”  One of their cases had multiple episodes after mild head trauma.  They noted that “transient blindness and hemiplegia may accompany the confusional state.”

Sakas, et al,[xi] wrote a paper in Neurosurgery in 1997 the purpose of which was to “explain the pathophysiology of the neurological deterioration that occurs after trivial head injures in children that is not caused by focal structural brain damage.”  They noted that the symptoms and/or signs included “headache, confusion, drowsiness, vomiting, hemiparesis, cortical blindness, and seizures.”  They noted that the assumption in this situation had been that underlying cerebral edema was responsible for these phenomena but this had been proven incorrect by cerebral imaging studies.  They proposed that:

children who are susceptible to such neurological attacks have an unstable ‘trigeminovascular reflex’ which is activated by craniofacial trauma…head trauma activates trigeminal nerve endings in the face, scalp, dura, or cortex, and via a reflex, causes intracranial vasodilation and cerebral hyperemia.

Soriani, et al,[xii] writing in 2000 in Archives of Pediatric and Adolescent Medicine on “Confusional Migraine Precipitated by Mild Head Trauma” reported on 8 boys and 3 girls, aged 6-14 years who presented after mild head trauma with confusion.  In addition to confusion their patients also experienced agitation (5), visual disturbances (3), bilateral mydriasis (3), dysarthria (1), somnolence (6), vomiting (7), and headache (6).  None of their patients had seizures and the attacks lasted from 1 to 12 hours.  They stated that the presence of a confusional state after head trauma often moved the treating physician to perform more extensive diagnostic testing such as CAT or MRI scanning of the brain and possible hospital admission.  They stressed that:

However, the possibility that confusion is caused by a migraine attack triggered by minor head trauma must be considered.

Borusiak[xiii] wrote in 2001 in Klin Padiatr about an 11 year old girl who presented with her first migrainous attack as a confusional state.  The diagnosis was made after the acute episode had subsided.  Katis writing in 2004[xiv] in Canadian Journal Emergency Medicine about acute confusional migraine (ACM) stated:

Children rarely present with auras, but often exhibit autonomic symptoms such as pallor, nausea, vomiting, and abdominal pain.  Discrete migraine equivalents or precursors have been observed from infancy to adolescence, in which headache is not prominent.

Katis stressed that confusional migraine is “a diagnosis of exclusion,” and the differential diagnoses should include “intracerebral injury, toxic ingestion, nonconvulsive seizure, post-ictal state, encephalitis, hypoglycemia, and metabolic derangement.”  He felt that if testing such as blood work, CT, and MRI scan are normal and ACM seems likely that “treatment consists of simple analgesics and sleep.”  He stressed that symptoms “normally resolve within several hours, and almost universally resolve within 24 hours.”

Dr. David Rothner[xv] reported on 90 cases of acute confusional migraine at the annual scientific meeting of the American Headache Society in 2007.  In his patients confusion lasted from 10 minutes to 2 days, with the majority of patients remaining confused for 4 hours or less, but 25 (28%) were confused between 5 and 8 hours.  All the children were disoriented, 72 had amnesia, 63 had speech impairment, 49 had agitation, 49 had emesis, 36 had visual disturbances, and 33 had somnolence.  Seventy four children had a family history of migraine and 52 had a personal history of migraine.  More than a third of his patients had recurrent acute confusional migraine events.  Boys aged 5-12 years, followed by boys aged 13-17 years, were most commonly affected.  Head trauma which often was very mild was present in more than a third of his cases.  Dr. Rothner described:

… a 14 year-old girl who experienced an aura followed by a bifrontal headache and a 5-hour period of progressive disorientation, confusion, incontinence, bizarre behavior, and extreme combativeness.  Once the confusion passed, the patient had no recollection of these events.  Her parents recalled two prior episodes that were less severe and involved nausea and vomiting.

Toxicology screens in 76 of Rothner’s patients were negative as were spinal fluid examinations in 29.  CT or MRI in 63 patients was normal in 57 and showed unrelated abnormalities in 6.  Electroencephalograms in 55 patients were abnormal in 44 with most showing unilateral or bilateral slowing.

Treatment

Katis[xvi] summarized treatment options with the following statement that “anti-migraine medications like sumatriptan, dihydroergotamine), metoclopramide, and prochlorperazine may be useful, as may prophylactic agents such as beta-blockers and calcium-channel blockers, but none of these agents have been systemically studied…”

 

[i] Livening, E.  Analytical table of cases.  On Megrim, Sick Headache, and Some Allied Disorders.  London:  J. and  A. Churchill, 1873.

[ii] Gascon G, Barlow C. Juvenile Migraine, Presenting As An Acute Confusional State.  Pediatrics.  1970;45 (4):628-635.

[iii] Whitty CWM.  Familial hemiplegic migraine.  J. Neurol. Neurosurg. Psychiat.  1953;16:172.

[iv] Selby G, and Lance JW.  Observations on 500 cases of migraine and Allied vascular headache.  J. Neurol. Neurosurg. Psychiat.  1960;23:23.

[v] Wolff  H.  Headache and Other Head Pain, ed.2 New York: Oxford University Press.  1963:342.

[vi] Gascon G, Barlow C. Juvenile Migraine, Presenting As An Acute Confusional State.  Pediatrics.  1970;45 (4):628-635.

[vii] Emery ES.  Acute Confusional State in Children With Migraine. Pediatrics.  1977; 60: 110-114.

[viii] Menken, M.  Transitory Confusion After Minor Head Injury.  Clinical Pediatrics.  1978;17:421-4.2.

[ix] Ehyai A, Fenichel GM. The Natural History of Acute Confusional Migraine.  Child Neurology.  1978;35:368-369.

[x] Ferrera PC, Reicho PR.  Acute confusional migraine and trauma-triggered migraine.  Am J Emerg Med.  1996;14(3):276-278.

[xi] Sakas DE, Whittaker KW, Whitwell HL, Singounas EG.  Syndromes of Posttraumatic Neurological.  Deterioration in Children with No Focal Lesions Revealed by Cerebral Imaging:  Evidence for a Trigeminovascular Pathophysiology.  Neurosurgery.  1997;41(3):661-667.

[xii] Soriana S, Cavaliere B, Faggioli R, Scarpa P, Borgna-Pignatti C.  Confusional migraine precipitated by hild head trauma.  Arch Pediatr Adolesc Med.   2000;154(1):90-91.

[xiii] Borusiak P.  First manifestation of migraine as acute confusional state:  “confusional migraine” and diagnostic problems.  Klin Padiatr.  2001;213(1):28-29.

[xiv] Katis PG.  Can J Emerg Med.  2004;6(6):451.

[xv] Rothner DA.  Cases Create Portrait of Confusional Migraine.  Clinical Neurology News.  March 2007:20.

[xvi] Katis PG.  Can J Emerg Med.  2004;6(6):451.