Pseudomigraine With Lymphocytic Pleocytosis: A Calcium Channelopathy? Clinical Description of 10 Cases and Genetic Analysis of the Familial Hemiplegic Migraine Gene CACNA1A

Abstract

Similarities between familial hemiplegic migraine and pseudomigraine with lymphocytic pleocytosis include recurrent headache with reversible neurologic deficit, cerebrospinal fluid lymphocytic pleocytosis, and triggers such as angiography. Even so, heteroduplex analysis and DNA sequencing failed to identify any sporadic mutations or shared polymorphisms in the exons or the intron/exon boundaries of the CACNA1A gene. These results do not support a role of the CACNA1A gene in the etiology of pseudomigraine with lymphocytic pleocytosis.

A Patient of Migraine-like Headache with Amnesia, Pleocytosis and Transient Hypoperfusion of Cerebral Blood Flow

“…In most patients with FHM, pleocytosis is observed (10), and such cases are difficult to differentiate from PMP. A recent study performed gene investigation in 8 patients in whom clinical findings suggested PMP, and reported that there were no abnormalities in the CACNAIA gene as observed in patients with FHM (11). In addition, diseases such as viral meningoencephalitis, granulomatous meningitis, neural syphilis, central infection with Borrelia or Mycoplasma, collagen disease-related angitis, cancerous meningitis, and Mollaret meningitis should be differentiated from PMP (2,3).…”

Sporadic hemiplegic migraine

“…Unlike SHM, viral-like premonitory symptoms occur before one third of HaNDL attacks, sufferers usually only endure one to 20 attacks over months before remission occurs, and only 18% involve visual symptoms whereas nearly every SHM attack includes visual aura [28]. One case of SHM with developmental delay, seizures, coma, and cerebellar and right hemispheric atrophy with ictal CSF pleocytosis has been associated with a CACNA1A mutation; another study screened 10 cases of HaNDL for CACNA1A mutations and found none [29,30]. Thus, differentiating SHM from HaNDL can be difficult, but attacks of HaNDL recur over less than 3 months in a monophasic period unlike SHM, which may recur for decades [3].…”

A HaNDL CASE with PAPILLEDEMA MIMICING TRANSIENT ISCHEMIC ATTACK

“…[3,5,[19][20][21] Chapman ve ark., 2003 yılında ailesel ve sporadik hemiplejik migren ile klinik benzerlikler gösteren HaNDL olgularında olası bir kanalopatiyi göstermek amacıyla CACNA1A gen mutasyonunu ve polimorfizmini taramış; ancak negatif sonuç almış-lardır. [22] Kürtüncü ve ark.nın 2012 yılında yaptığı bir çalışma ile HaNDL sendromlu iki hastada T-tipi kal-PAIN A RI siyum kanal alt birimlerinden CACNA1H proteinine karşı yüksek titrede antikorlar saptanmış, iyon kanalı otoimmünitesinin sendromun patogenezine kısmen de olsa katkı sağlayabileceği görüşü desteklenmiştir. [23] HaNDL sendromunun patogenezi halen bilinmediğinden bu alanda çalışmalara gerek vardır ve ayrın-tılı olgu bildirimleri de değerlidir.…”