&lt;p&gt;Chorea and the effectiveness of steroids in a patient with the syndrome of transient headache with neurologic deficits and cerebrospinal fluid lymphocytosis: a case report&lt;/p&gt;

Zhao, Lu; Wang, Ruihao; Fang, Hui; Song, Bo; Liang, Dongyi; Xu, Yuming

doi:10.2147/jpr.s204869

Cited by 8 publications

(6 citation statements)

References 19 publications

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“…There are currently no treatment recommendations for HaNDL. However, symptomatic treatment has been applied previously in cases with severe symptomatology: acetazomide to reduce abnormally high intracranial pressure [ 14 ], steroidal treatment given against a possible immune-mediated reaction after an infection or other inflammatory triggers [ 1 , 15 ], and treatment with valproic acid thought to affect the possible pathophysiological mechanism of cortical spreading depression (CSD) similar to migraine [ 16 ]. Moreover, it is probable that less-known features of valproic acids, such as the capacity to diminish replication of enveloped viruses and anti-inflammatory action through the modulation of innate and adaptive immunity cells [ 17 ], could contribute to a beneficial outcome.…”

Section: Discussionmentioning

confidence: 99%

Syndrome of Transient Headache and Neurologic Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): HHV-7 Finding in Cerebrospinal Fluid Challenges Diagnostic Criteria

2023

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The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a rare, self-limiting condition with severe headaches combined with neurological symptoms. However, evidence-based recommendations on diagnostics and treatments are unavailable due to the condition’s rarity and unknown pathophysiology. A young man experiencing severe headache attacks fulfilled the HaNDL diagnostic criteria according to the third edition of the International Classification of Headache Disorders (ICHD-3). We present the dynamics of cerebrospinal fluid (CSF) biomarkers related to low human herpesvirus 7 (HHV-7) load and anti-inflammatory treatment outcomes. Low HHV-7 load may be an immunological trigger of HaNDL, such that elevated levels of CSF- chemokine (C-X-C motif) ligand 13 open a new way to interpret the role of B cells in HaNDL pathogenesis. We discuss the diagnostic challenge of HaNDL, according to the ICHD-3, in the case of pathogen presence at low load in CSF.

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Section: Discussionmentioning

confidence: 99%

Syndrome of Transient Headache and Neurologic Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): HHV-7 Finding in Cerebrospinal Fluid Challenges Diagnostic Criteria

2023

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“…Follow-up CSF analysis was reported in 23 cases, with universal reduction of lymphocytic pleocytosis associated with resolution of transient neurologic deficits. The degree of reduction varied significantly, as did the time period over which the reduction occurred (1,3,(17)(18)(19)(20)23,24,26,29,31,34,35,43,(47)(48)(49)53,56,(60)(61)(62)65).…”

Section: Csf Characteristicsmentioning

confidence: 99%

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis syndrome: A comprehensive systematic review of 93 patients from 57 studies

et al. 2023

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Background Headache with neurologic deficits and cerebrospinal fluid lymphocytosis, previously also termed pseudomigraine with temporary neurologic symptoms and lymphocytic pleocytosis, is a self-limiting syndrome characterized by moderate to severe headache associated with focal neurological deficits occurring in the context of lymphocytosis in the cerebrospinal fluid. As a consequence of its rarity, data regarding headache with neurologic deficits and cerebrospinal fluid lymphocytosis is sparse. Therefore, we conducted this review to analyze data related to 93 patients of headache with neurologic deficits and cerebrospinal fluid lymphocytosis, to characterize their demographics, clinical manifestations, investigations and treatment options. Methods We performed a systematic review of cases reported through PubMed and Google scholar database, using Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol. Keywords used were ‘Headache with Neurologic Deficits and cerebrospinal fluid lymphocytosis’, ‘Headache with neurologic deficits and cerebrospinal fluid lymphocytosis syndrome’. The quality of the included studies was assessed using the Joanna Briggs Institute Critical Appraisal Tool. Results We analyzed a total of 93 cases of headache with neurologic deficits and cerebrospinal fluid lymphocytosis with a mean age of 28.8 years at onset. Seventy patients (75.2%) were adults, while 23 (24.7%) belonged to the pediatric age group. Comparing these groups, mean age at onset was 32.5 years and 14.3 years, respectively. The average duration of follow-up was 11.08 months. Thirty percent of patients experienced relapsing episodes of headache with neurologic deficits and cerebrospinal fluid lymphocytosis symptoms. The most common type of headache reported was unilateral severe throbbing episodic headache. Other associated symptoms included sensory deficit (60%) and motor deficits (54.8%). The least common symptoms were nystagmus and agraphia, which were reported in one patient each. Antiviral agents were a common treatment option in the acute phase (n = 23 patients [23.6%]), while Flunarizine was the most commonly used agent in the chronic setting (n = 3 patients [3.2%]). While most of the patients had normal brain magnetic resonance imaging, 20 patients had magnetic resonance imaging abnormalities, including (but not limited to) non-specific white matter lesions (eight patients) and meningeal enhancement (six patients). The most common electroencephalographic findings included diffuse and focal slowing. The mean cerebrospinal fluid opening-pressure was 240.5 mmH2O. Cerebrospinal fluid protein was elevated in 59 (63.4%) patients, with a mean value of 114 mg/dL. Two patients in our cohort were found to have cerebrospinal fluid oligoclonal bands. Conclusion Headache with neurologic deficits and cerebrospinal fluid lymphocytosis tends to affect young individuals with a slight male predominance. Unilateral severe throbbing episodic headache with associated hemi-paresthesia and hemiparesis were the most common symptoms based on our review. Elevated cerebrospinal fluid opening-pressure can be seen in headache with neurologic deficits and cerebrospinal fluid lymphocytosis syndrome. Early recognition of the syndrome is paramount. Antivirals were found to be among the most widely used treatments in the acute setting. Magnetic resonance imaging of the brain is mostly normal. Diffuse and focal slowing were among the most common electroencephalographic findings. Cerebral flow abnormalities on perfusion scans are not uncommon in headache with neurologic deficits and cerebrospinal fluid lymphocytosis. Prospective studies with a larger sample size are needed to validate our findings and guide the clinical care of these patients.

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“…Methylprednisolone acetazolamide has been reported as a treatment, especially when an association of HaNDL and intracranial hypertension was detected. [3][4][5][6] Prednisolone, valproic acid, and verapamil were used together in one case, but the effectiveness of these drugs was not mentioned in detail. 7 We have prescribed oral Valproic acid 1000-1500 mg for an average of 1-4 months.…”

Section: Patientmentioning

confidence: 99%

Case series of HaNDL syndrome responding to valproic acid

Aydınlar

Ertaş

2022

Cephalalgia Reports

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Objective: We report a case series of patients diagnosed with the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) responding to high dose Valproic acid. Background: HaNDL syndrome is an infrequent entity, presenting with neurological impairment episodes and severe headache. A treatment to prevent the attacks has not been proposed yet. Results: We describe 6 patients with a definite diagnosis of HaNDL, responding to Valproic acid 1000–1500 mg/day. Conclusion: Although HaNDL’s self-limiting nature, episodes may cause important disability and can last up to 3 months. Valproic acid may be a good choice to prevent attacks due to its effect on cortical depression and fast titration.

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<p>Chorea and the effectiveness of steroids in a patient with the syndrome of transient headache with neurologic deficits and cerebrospinal fluid lymphocytosis: a case report</p>

Cited by 8 publications

References 19 publications

Syndrome of Transient Headache and Neurologic Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): HHV-7 Finding in Cerebrospinal Fluid Challenges Diagnostic Criteria

Syndrome of Transient Headache and Neurologic Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): HHV-7 Finding in Cerebrospinal Fluid Challenges Diagnostic Criteria

Transient headache and neurological deficits with cerebrospinal fluid lymphocytosis syndrome: A comprehensive systematic review of 93 patients from 57 studies

Case series of HaNDL syndrome responding to valproic acid

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