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

Tada, Yukiko; Negoro, Kiyoshi; Abe, Masaaki; Ogasawara, Jun Ichi; Kawai, Motoharu; Morimatsu, Mitsunori

doi:10.2169/internalmedicine.44.743

Cited by 15 publications

(9 citation statements)

References 14 publications

(10 reference statements)

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“…The syndrome is characterised by (A) moderate to severe headaches with one or more transient neurological deficits lasting several hours, (B) the disappearance of attacks within 2 months, (C) a CSF lymphocytic pleocytosis (>15 white cells per μL), with negative reaction on bacteriological, viral and fungal test, (D) the absence of neuroradiological abnormalities other than transient decrease in cerebral blood flow and (E) the absence of epileptic waves on electroencephalographs 4 5. Focal neurological deficits are restricted to one hemisphere in most patients2 and include hemiparaesthesia in three quarters of cases, dysphasia in half and hemiparesis in a little over half of patients, which usually last over 4 h (some up to 24 h) without leaving neurological sequelae and with symptom-free intervals 6.…”

Section: Discussionmentioning

confidence: 99%

“…Papilloedema is occasionally present 7 9. Atypical presentations such as transient global amnesia,4 and confusion with decreased level of consciousness in paediatric cases8 10 and adult cases11 have been reported, which may blur diagnostic clarity.…”

Section: Discussionmentioning

confidence: 99%

“…HaNDL syndrome is a benign and self-limiting disorder usually observed in young adults between 15–40 years of age,5 6 and has a higher incidence in men, contrary to the incidence of migraine, which is more common in women 4 6…”

Section: Discussionmentioning

confidence: 99%

“…An infectious hypothesis states that an infective process (probably viral) may trigger antibodies directed against neuronal or neurovascular entities such as the trigeminal terminal of the pachymeninx blood vessels,3 or cause an aseptic leptomeningeal vasculitis,8 resulting in transient vascular headaches and neurological symptoms via mechanisms resembling cortical spread depression 4 6 11. Most patients report a prodrome of a viral-like illness, with 25% reporting this within 3 weeks prior to symptom onset 6 8.…”

Section: Discussionmentioning

confidence: 99%

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HaNDL syndrome in a 14-year-old girl

2016

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Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) consists of recurrent headaches with focal neurological signs, which can include motor, sensory and aphasic symptoms. Although considered rare, it is becoming increasingly recognised in clinical practice due to the accumulation of case reports. The pathophysiology remains unclear although changes in the neurovascular resemble those found in migraine, which are thought to be triggered by an infectious process. HaNDL can mimic various serious, including life-threatening, diseases, such as stroke and meningoencephalitis, which is why vigorous tests should be sought before this diagnosis of exclusion can be reached. Treatment is symptomatic and the prognosis is excellent. A literature review of the topic is discussed. We report an adolescent girl who presented with recurrent expressive dysphasia and right-sided hypoaesthesia and moderate occipital headaches who was diagnosed with HaNDL syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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HaNDL syndrome in a 14-year-old girl

2016

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“…2016;7(3):84-86 young adults (15 -40 years) and is characterized by recurrent episodes (1 to more than 15) of headache and neurological deficits that last from a few hours to 3 days and are separated by symptom-free intervals [2]. Unlike migraine, it has male predominance, a lack of personal or familiar history of migraine and a prodrome of viral infection in about one-third of the cases [4]. Etiology of HaNDL is not yet fully understood.…”

Section: Discussionmentioning

confidence: 99%

How to HaNDL This Case?

et al. 2016

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Headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytosis (HaNDL) is a self-limited, benign neurological entity that may constitute a diagnosis dilemma since it can mimic a variety of conditions including aseptic meningitis, meningoencephalitis, migraine with aura and stroke. We report a case of a 31-year-old Caucasian man admitted at the emergency department for sudden onset of dysarthria and paresthesias in the right upper limb, after 2 weeks of complaints of headache associated with mild nausea. After extensive blood investigation, CSF study, electroencephalogram and neuroimaging, central nervous system infections, autoimmune and vascular diseases were excluded. CSF study revealed lymphocytic pleocytosis, normal glucose and slightly elevated protein concentration. Magnetic resonance imaging (MRI) showed a diffuse leptomeningeal enhancement, a decreased and delayed perfusion to the entire left hemisphere. The patient improved with supportive and symptomatic management. Two days after discharge, he developed a similar clinical picture and recovered completely 30 minutes after admission. His repeated lumbar puncture (LP) showed progressive improvement as expected due to the clinical resolution and MRI did not reveal any of the previous changes. Given the presentation (clinical picture, imaging and CSF studies), the subsequent similar and self-limited episode and having excluded other causes, a final diagnosis of HaNDL was made. HaN-DL is one of the most frequent stroke mimics and probably not as rare as commonly thought. It has a good prognosis since this monophasic, self-limiting condition runs its course usually within a few months from onset, without any neurological sequelae.

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