Symptoms of definite persistent aura

b c c runner [subject #73], Visual snow, 2006. © 2006 b c c runner [more]

Helmut [subject #296], Visual snow and floaters, 2007. © 2007 Helmut [more]

Persistent symptoms

In the subgroup of 60 subjects (#1, #8, #14, #20, #23, #30, #42, #45, #52, #73, #75, #80, #85, #86, #104, #120, #136, #138, #141, #142, #145, #147, #152, #153, #156, #157, #159, #162, #167, #168, #169, #171, #172, #175, #179, #180, #182, #185, #189, #200, #217, #228, #230, #233, #234, #235, #240, #254, #257, #258, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301) with a diagnosis of definite persistent aura without infarction, the most frequently reported persistent symptoms were visual hallucinations of random form dimension (51 subjects), chronic tinnitus (25 subjects), visual loss (20 subjects), visual perseveration (18 subjects), hypersensitivity to light (12 subjects) or sound (3 subjects), hypersensitivity to visual discomfort (11 subjects), vertigo, dizziness or other vestibular sensations (10 subjects), cognitive problems (10 subjects), increased halos (8 subjects), derealisation (8 subjects), impaired night vision (7 subjects), visual reading problems (7 subjects), autokinesis (5 subjects), monocular diplopia (4 subjects), increased starbursts (4 subjects), hypacusia (4 subjects), disturbances of visual processing (3 subjects), muscle twitching (3 subjects), bilateral somatosensory symptoms (2 subjects), depersonalisation (3 subjects), problems maintaining focus (2 subjects), disturbance of depth perception (2 subjects), visual hallucinations of line form dimension (2 subjects), lattice form dimension (2 subjects) or kaleidoscope form dimension (2 subjects), synaesthesia (2 subjects), binocular diplopia (2 subjects), corona phenomenon (2 subjects), hyperawareness of reflections, shadows or one's own body parts (2 subjects), musical palinacusis (2 subjects), olfactory hallucinations (2 subjects), hyposmia (2 subjects) or aphasic disturbances (2 subjects), respectively. Further varieties of persistent symptoms were only observed in single cases (see Table).

It is important to note that this list of persistent aura symptoms includes 7 symptoms which are known to occur as transitory aura symptoms in basilar-type migraine: visual symptoms (i.e. visual hallucinations of random form dimension) simultaneously in both temporal and nasal fields of both eyes (51 subjects [#1, #8, #14, #20, #23, #30, #42, #45, #52, #73, #75, #86, #104, #120, #136, #138, #141, #142, #145, #147, #156, #159, #162, #169, #171, #172, #175, #179, #182, #185, #189, #200, #217, #228, #230, #233, #234, #235, #240, #254, #257, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301]), tinnitus (25 subjects [#42, #45, #52, #73, #75, #86, #104, #120, #138, #153, #157, #162, #167, #171, #179, #189, #200, #217, #228, #230, #235, #240, #273, #284, #296]), vertigo or other vestibular symptoms (10 subjects [#23, #45, #52, #86, #142, #162, #172, #185, #234, #277]), diplopia (4 subjects [#8, #157, #179, #234] with monocular and 2 subjects [#172, #189] with binocular diplopia), hypacusia (4 subjects [#153, #189, #200, #234]), bilateral somatosensory symptoms (2 subjects [#189, #230]) and ataxia (1 subject [#172]), respectively.

Overall, 54 or 90,0 % of the 60 subjects with a diagnosis of definite persistent aura without infarction had at least one of these basilar-type persistent aura symptoms (subjects #1, #8, #14, #20, #23, #30, #42, #45, #52, #73, #75, #86, #104, #120, #136, #138, #141, #142, #145, #147, #153, #156, #157, #159, #162, #167, #169, #171, #172, #175, #179, #182, #185, #189, #200, #217, #228, #230, #233, #234, #235, #240, #254, #257, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301). Of these 54 subjects, 46 subjects (#20, #23, #42, #45, #52, #73, #75, #86, #104, #120, #138, #142, #145, #153, #156, #157, #159, #162, #167, #169, #171, #172, #175, #179, #182, #185, #189, #200, #217, #230, #233, #234, #235, #240, #254, #257, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301) reported one or more transitory symptoms of basilar-type migraine (see below) and 31 subjects (#23, #42, #45, #52, #75, #86, #104, #120, #156, #157, #159, #162, #167, #169, #171, #179, #185, #189, #200, #230, #233, #235, #254, #257, #264, #265, #276, #284, #296, #283, #301) fully matched the diagnostic criteria of basilar-type migraine with two (subjects #104, #159, #167, #179, #185, #189, #233, #235, #254, #257, #264, #276, #283, #296), three (subjects #52, #75, #86, #156, #200, #265, #284, #301), four (subjects #42, #169, #171, #230), five (subjects #120, #162), six (subject #45) or seven (subjects #23, #157) different basilar-type aura symptoms.

In the sample of participants in Sofia Greene's internet survey who got a diagnosis of definite persistent aura without infarction, the condition clearly appears to be a complication of basilar-type migraine in the majority of subjects. These data considerably expand previous reports of persistent basilar-type migraine aura symptoms that focused on the symptom of decreased level of consciousness (Lee and Lance, 1977; Frequin et al., 1991; Muellbacher and Mamoli, 1994). One must be careful, however, to generalize these observations to all cases of persistent aura without infarction, given the present study's focus on persisting visual disturbances (which are often bilateral) which may have favoured the inclusion of sufferers from basilar-type persistent migraine aura symptoms.

On the other hand, it is worth mentioning that the distribution of persistent symptoms encountered in the present study agrees with Randolph W. Evans' (2002) textbook description (in major parts based on Liu et al., 1995) of the "usual" clinical presentation of persistent aura without infarction, suggesting that our large sample may well be representative of the given disorder in general:

"d. Rarely, migraineurs have persistent visual aura.
(1) Usually simple, unformed hallucinations in the entire visual field of both eyes may be described as a million dots, television static, clouds, dots, heat waves, flashing or flickering lights, lines of ants, rainlike pattern, snow, squiggles, bubbles, and grainy vision.
(2) Occasionally, palinopsia (the persistence of visual images), micropsia, or formed hallucinations.
(3) Might respond to preventive treatment with divalproex sodium."

Highlighting the importance of basilar-type migraine, which is defined as migraine with aura symptoms clearly originating from the brainstem and/or from both hemispheres simultaneously affected, but no motor weakness (see here), the results from Sofia Greene's internet survey provide clinical evidence of a persistent dysfunction in the brain stem in the majority of sufferers from persistent aura without infarction. Activation in the brain stem during attacks of migraine has been detected with the use of functional imaging, suggesting an important role of the brain stem in this disorder (Weiller et al., 1995; Afridi et al., 2005). The results from Sofia Greene's internet survey are consistent with these previous studies indicating an important role of the brain stem in migraine (Wieser et al., 2004).

Transitory symptoms

Gyps Kindra [subject #120], Zigzagged fortification spectrum, 2006. © 2006 Gyps Kindra [more]

All 60 subjects (#1, #8, #14, #20, #23, #30, #42, #45, #52, #73, #75, #80, #85, #86, #104, #120, #136, #138, #141, #142, #145, #147, #152, #153, #156, #157, #159, #162, #167, #168, #169, #171, #172, #175, #179, #180, #182, #185, #189, #200, #217, #228, #230, #233, #234, #235, #240, #254, #257, #258, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301) with persistent aura symptoms also reported a history of transitory aura symptoms before (48 subjects [#1, #8, #14, #20, #23, #30, #42, #45, #52, #73, #75, #80, #85, #86, #104, #136, #141, #142, #145, #153, #156, #157, #159, #162, #167, #168, #169, #180, #182, #200, #228, #230, #233, #234, #240, #254, #257, #258, #264, #265, #273, #277, #283, #284, #300, #301]) and/or after (46 subjects [#1, #8, #14, #20, #23, #42, #45, #52, #73, #75, #85, #86, #104, #120, #141, #145, #147, #156, #157, #159, #162, #167, #169, #171, #172, #175, #179, #185, #189, #200, #217, #228, #230, #233, #235, #240, #254, #257, #273, #276, #277, #283, #284, #296, #300, #301]) the onset of the 1st episode of persistent aura.

Jane10 [subject #23], Persistent migraine aura, 2006. © 2006 Jane10 [more]

Jane10 [subject #23], Onset of attack of migraine with aura occurring in addition to persistent migraine aura, 2006. © 2006 Jane10 [more]

Jane10 [subject #23], Right-sided homonymous hemianopia developing in the course of an attack of migraine with aura occurring in addition to persistent migraine aura, 2006. © 2006 Jane10 [more]

Fourty-eight subjects (#20, #23, #42, #45, #52, #73, #75, #85, #86, #104, #120, #138, #142, #145, #153, #156, #157, #159, #162, #167, #169, #171, #172, #175, #179, #180, #182, #185, #189, #200, #217, #230, #233, #234, #235, #254, #257, #258, #264, #265, #273, #276, #277, #283, #284, #296, #300, #301) described one or more (mean 2,43, range 1-7) aura symptoms characteristic of basilar-type migraine, 17 subjects (#14, #23, #45, #52, #75, #85, #86, #120, #141, #145, #156, #169, #171, #175, #228, #300, #301) one or more (mean 1,29, range 1-2) aura symptoms characteristic of typical aura with migraine headache, 3 subjects (#14, #23, #284) 3 or more (mean 3,33, range 3-4) aura symptoms characteristic of sporadic hemiplegic migraine and 1 subject (#145) fully reversible monocular positive visual phenomena (scotomas) characteristic of retinal migraine. Thirty-three subjects (#23, #42, #45, #52, #75, #85, #86, #104, #120, #156, #157, #159, #162, #167, #169, #171, #179, #180, #185, #189, #200, #230, #233, #235, #254, #257, #264, #265, #276, #283, #284, #296, #301) fulfilled the IHC ICHD-II criteria of basilar-type migraine, 14 subjects (#14, #23, #52, #75, #85, #86, #120, #141, #145, #156, #169, #171, #175, #228) of typical aura with migraine headache, 3 subjects (#14, #23, #284) of sporadic hemiplegic migraine and 1 subject (#145) of retinal migraine, respectively.

The various transitory aura symptoms experienced by the persistent aura sufferers are summarized in the table below and presented in more detail here.

References

Afridi SK, Griffin NJ, Kaube H, Friston KJ, Ward NS, Frackowiak RS, Goadsby PJ. A positron emission tomographic study in spontaneous migraine. Arch Neurol 2005; 62: 1270-1275.
Frequin ST, Linssen WH, Pasman JW, Hommes OR, Merx HL. Recurrent prolonged coma due to basilar artery migraine. A case report. Headache 1991; 31: 75-81.
Lee CH, Lance JW. Migraine stupor. Headache 1977; 17: 32-38.
Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology 1995; 45: 664-668.
Muellbacher W, Mamoli B. Prolonged impaired consciousness in basilar artery migraine. Headache 1994; 34: 282-285.
Weiller C, May A, Limmroth V, Juptner M, Kaube H, Schayck RV, Coenen HH, Diener HC. Brain stem activation in spontaneous human migraine attacks. Nature Med 1995; 1: 658-660.
Wieser T, Wolff R, Hoffmann KP, Schulte-Mattler W, Zierz S. Persistent ocular motor disturbances in migraine without aura. Neurol Sci 2004; 25: 8-12.

Acknowledgments: We wish to thank SwedeP (subject #44) for providing us with a review of the symptomatology of visual snow sufferers at an early stage of our study.

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