CACNA1H antibodies associated with headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL)

Kürtüncü, Murat; Kaya, Dilaver; Zuliani, Luigi; Erdağ, Ece; İçöz, Sema; Uğurel, Elif; Çavuş, Filiz; Ayşit, Neşe; Birişik, Ömer; Vincent, Angela; Eraksoy, Mefküre; Vural, Burçak; Akman‐Demir, Gülşen; Tüzün, Erdem

doi:10.1177/0333102412463494

Cited by 42 publications

(31 citation statements)

References 11 publications

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“…Some patients report a preceding non-specific viral prodromal illness [6,14], but studies have failed to provide a definite viral association [2,3]. The presence of antibodies directed against the CACNA1H subunit of the T-type voltage-gated calcium channel in one small study supported the inflammatory or autoimmune hypothesis [15]. Cortical spreading depression-associated cerebral oligaemia has been observed during HaNDL attack, and may play a significant role [8,16].…”

Section: Discussionmentioning

confidence: 98%

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (Handl Syndrome): A case report

et al. 2017

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The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related with migrainous headaches. It is a benign disorder, self-limited, and characterized by fluctuating neurological symptoms and cerebrospinal fluid (CSF) lymphocytosis. A small proportion of patients develop intracranial hypertension as a consequence of the illness. Confusional state may be also observed.We describe a case of a 29 years old man with acute onset of headache, dysthermia sensation, vomits, aphasia, and difficulty to perform daily activities such as unlocking the mobile phone. Blood tests as well as computed tomography (CT) and magnetic resonance imaging (MRI) were normal. Cerebroespinal fluid analysis revealed a lymphocytic pleocytosis (47 cells/µl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with frontotemporal predominance, and without epileptiform activity. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged asymptomatic without treatment.

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Section: Discussionmentioning

confidence: 98%

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (Handl Syndrome): A case report

et al. 2017

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“…Specifically, a recent study has demonstrated autoantibodies to subunit of the T-type voltage-gated calcium channel, CACNA1H, has been linked to HaNDL syndrome (8). In addition, mitogen activated protein kinase-4 (MAPK-4) and DNA-dependent protein kinase catalytic subunit (DPKCU) antibodies were also detected in minority of HaNDL patients (8,9).…”

Section: Discussionmentioning

confidence: 99%

Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis masquerading as meningitis in HIV patient

et al. 2017

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Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) is described as "headache attributed to noninfectious inflammatory disease" featuring, as its name suggests, headache that mimics migraine in addition to neurologic symptoms such as hemiparesis, hemiparesthesia and dysphagia. We report a case of a 50-year-old African-American female who presented with headache, malaise and subsequent hemiparesis. Despite bearing a close resemblance to an acute episode of meningitis clinically, cerebrospinal fluid (CSF) analysis of the patient was only positive for isolated elevation in white cell count. The patient was diagnosed with HaNDL syndrome which is characterized by transient headache and neurologic deficits with CSF lymphocytosis. While the overall condition often appears substantial, the disease is self-limiting and patients usually recover spontaneously.

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“…[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.…”

Section: Discussionunclassified

A HaNDL CASE with PAPILLEDEMA MIMICING TRANSIENT ISCHEMIC ATTACK

Gungor

Çakar²,

Orhan³

et al. 2016

Agri

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ÖzetBeyin omurilik sıvısı (BOS) lenfositozu ve geçici nörolojik bulguların eşlik ettiği baş ağrısı sendromu ('The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis: HaNDL') nadir görülen, iyi seyirli, kendini sınırlayan ve az tanınan bir sendromdur. Zengin nörolojik semptomatolojisi ile morbidite ve mortaliteye yol açan çok sayıda hastalığı taklit edebilen bu sendrom için, klinik şüphe ile yapılan BOS incelemesi tanısal değer taşır. Etiyopatogenezi tam olarak bilinmese de, otoimmünite ön planda düşünülmektedir. Bu yazıda, ilk klinik prezentasyonu geçici iskemik atağı taklit eden, geçici ve tekrarlayıcı nörolojik bulgular gösteren epizodları ve normal BOS basıncına eşlik eden papilödem bulgusu ile bir HaNDL olgusu tartışıldı, sendromun tanımı, klinik tablo ve epidemiyolojisi, etiyopatogenezi, ayırıcı tanısı ve tedavisi literatür eşliğinde gözden geçirildi.Anahtar sözcükler: HaNDL, inme taklidi; intravenöz trombolitik tedavi; lenfositik pleositoz; otoimmün hastalık. SummaryThe syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL), is a rare, benign, self-limiting syndrome that is not well known. Cerebrospinal fluid examination (CSF) on clinical suspicion has diagnostic value for this syndrome, which has a rich neurological symptomatology that can mimic many diseases causing mortality and morbidity. Although the ethiopathogenesis of this syndrome is not fully known, autoimmunity is thought to be in the foreground. Described in this article is a HaNDL case that on first clinic presentation appeared to be a transient ischemic attack (TIA) with temporary but recurring neurological findings and normal CSF pressure accompanied by papilledema. Clinical features, epidemiology, ethiopathogenesis, differential diagnosis, and treatment of this syndrome are reviewed according to the literature.

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CACNA1H antibodies associated with headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL)

Abstract: Our results support the notion that ion channel autoimmunity might at least partially contribute to HaNDL pathogenesis and occurrence of neurological symptoms.

Cited by 42 publications

References 11 publications

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (Handl Syndrome): A case report

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (Handl Syndrome): A case report

Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis masquerading as meningitis in HIV patient

A HaNDL CASE with PAPILLEDEMA MIMICING TRANSIENT ISCHEMIC ATTACK

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