Headache And Neurological Deficits With Cerebrospinal Fluid Lymphocytosis Research Articles

Transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) following COVID-19.

An abstract is not available for this content. As you have access to this content, full HTML content is provided on this page. A PDF of this content is also available in through the ‘Save PDF’ action button.

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

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.

Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): A Case Report - Is there an Association with the COVID-19 Vaccine?

Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): A Case Report - Is there an Association with the COVID-19 Vaccine?

Case series of HaNDL syndrome responding to valproic acid

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.

A case report on Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL)

HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is a rare headache disorder previously known as Migraine with cerebrospinal fluid pleocytosis. The disease is characterized by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. It is a benign disorder and a stroke mimicker. We describe a case of 50 years old lady presented with acute onset headache, right hemi sensory tingling and bilateral papilloedema. Her blood test, MRI of brain and MRV was normal. CSF study revealed lymphocytic pleocytosis. The patient was discharged with full recovery. HaNDL syndrome is a diagnosis of exclusion. High degree of suspicion and characteristic clinical and laboratory findings are important to recognize.
 J Dhaka Medical College, Vol. 29, No.1, April, 2020, Page 89-91

The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL): a description of three patients

Three clinical cases of the syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) are first described in Russian literature. The patients were young (aged 30–35 years), had symptoms characteristic of the prodromal period of infections. In all the patients, the disease started with intense headache, followed by focal neurological symptoms: aphasia and hemihypesthesia in all cases and confusion with psychomotor agitation in two cases. All the three patients showed spontaneous recovery within 2–3 days. Perfusion computed tomography, magnetic resonance imaging, and electroencephalography are compared in one of the cases. The differential diagnosis of HaNDL with acute cerebrovascular accident, herpetic encephalitis, epilepsy, and migraine is discussed.

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.

BackgroundThe clinical manifestations of the transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) syndrome are variable, and involuntary movements have not been reported. The etiology and treatment of the syndrome are not entirely clarified.CaseA 25-year-old female presented with recurrent transient headache, involuntary movements (left upper extremity chorea) and paralysis. Lumbar punctures showed intracranial hypertension and cerebrospinal fluid pleocytosis. Symptoms and intracranial hypertension were relieved after administration of steroids.Discussion/conclusionChorea may also present in the HaNDL syndrome. Steroids might relieve the symptoms of the HaNDL syndrome, which indicates the possible treatment strategy for HaNDL and supports the infectious or postinfectious autoimmune etiology hypothesis.

Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): A Case Report with Newly Described Electroencephalography (EEG) findings, Symptomatic Treatment Strategies, and Literature Review (P5.10-008)

Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): A Case Report with Newly Described Electroencephalography (EEG) findings, Symptomatic Treatment Strategies, and Literature Review (P5.10-008)

Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL)

The authors make a narrative review on "headache syndrome and transient neurological deficits with cerebrospinal fluid lymphocytosis (CRL)," a rare, underdiagnosed disorder of little knowledge by physicians specializing in care in the emergency room. Aspects of the clinical picture, diagnosis, prognosis, and treatment are discussed

Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL)

Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL)

Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL)

Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL)

Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL): A case report with serial electroencephalography (EEG) recordings.. Is there an association with human herpes virus type 7 (HHV-7) infection?

Introduction The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a diagnosis made by exclusion. In the literature, different etiological explanations are proposed for HaNDL, including an immune-mediated reaction after a viral infection. Case description We present a case of a 23-year-old woman with several episodes of transient headache, neurological deficits and cerebrospinal fluid lymphocytosis. All diagnostic criteria for the HaNDL syndrome were fulfilled; however, additional cerebrospinal fluid analysis showed a positive polymerase chain reaction (PCR) for human herpes virus type 7 (HHV-7). Discussion The possible role of a (prodromal) viral infection in the etiology of HaNDL is discussed. Also the role of electroencephalography (EEG) recordings is discussed. Serial EEG recordings showed generalized slowing, frontal intermittent rhythmic delta activity (FIRDA) and symmetric triphasic frontal waves with a dilation lag.

HaNDL syndrome: Correlation between focal deficits topography and EEG or SPECT abnormalities in a series of 5 new cases

IntroductionTransient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) is characterised by migraine-like headache episodes accompanied by neurological deficits consisting of motor, sensory, or aphasic symptoms. Electroencephalogram (EEG) and single photon emission computed tomography (SPECT) may show focal abnormalities that correspond to the neurological deficits. We aim to evaluate the correlation between focal deficit topography and EEG or SPECT abnormalities in 5 new cases. PatientsWe retrospectively reviewed patients attended in a tertiary hospital (January 2010-May 2014) and identified 5 patients (3 men, 2 women) with a mean age of 30.6±7.7 (21-39) years. They presented 3.4±2.6 episodes of headache (range, 2-8) of moderate to severe intensity and transient neurological deficits over a maximum of 5 weeks. Pleocytosis was detected in CSF in all cases (70 to 312cells/mm3, 96.5-100% lymphocytes) with negative results from aetiological studies. ResultsAt least one EEG was performed in 4 patients and SPECT in 3 patients. Patient 1: 8 episodes; 4 left hemisphere, 3 right hemisphere, and 1 brainstem; 2 EEGs showing left temporal and bilateral temporal slowing; normal SPECT. Patient 2: 2 episodes, left hemisphere and right hemisphere; SPECT showed decreased left temporal blood flow. Patient 3: 3 left hemisphere deficits; EEG with bilateral frontal and temporal slowing. Patient 4: 2 episodes with right parieto-occipital topography and right frontal slowing in EEG. Patient 5: 2 episodes, right hemisphere and left hemisphere, EEG with right temporal slowing; normal SPECT. ConclusionThe neurological deficits accompanying headache in HaNDL demonstrate marked clinical heterogeneity. SPECT abnormalities and most of all EEG abnormalities were not uncommon in our series and they did not always correlate to the topography of focal déficits.

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis mimicking an acute stroke.

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) can present as sudden onset of focal neurological deficits which are clinically and radiologically indistinguishable from an ischaemic stroke. Its diagnosis requires a lumbar puncture (LP), which contraindicates intravenous thrombolytic therapy (IV-tPA). All patients referred to our stroke centre as a stroke code resulting in a final diagnosis of HaNDL syndrome from June 2005 to June 2015 were retrospectively analysed. Nine cases were identified: seven women and two men (mean age 27.6 years, range 15-51). Clinical onset consisted of isolated aphasia (two) and aphasia with right hemiparesis/hemiparaesthesia (seven). All patients had headache in the acute setting, lasting 2-12 h. Cranial computed tomography (CT) and CT angiography (CTA) were normal in all patients. Perfusion CT was performed in seven patients, showing left hemispheric focal hypoperfusion in five cases; the remaining two were normal. Five patients were initially diagnosed as stroke and treated uneventfully with IV-tPA. Cranial magnetic resonance imaging within 48 h was normal in all cases. LP performed in all patients showed pleocytosis (range 17-351 cells/mm(3) ), high protein levels (range 0.4-1.6 g/l) and normal glucose levels. All cases recovered within 12 h and suffered a second episode within 72 h. Patients were asymptomatic between episodes and after remission. The decision to thrombolyse or perform an LP in HaNDL patients mimicking a stroke is difficult in the acute setting. Perfusion CT can provide misleading results and CTA may be useful in ruling out occlusion of a cerebral vessel.

Síndrome HaNDL: correlación entre la topografía del déficit neurológico y las alteraciones en electroencefalograma y SPECT en una serie de 5 nuevos casos

IntroductionTransient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) is characterised by migraine-like headache episodes accompanied by neurological deficits consisting of motor, sensory, or aphasic symptoms. Electroencephalogram (EEG) and single photon emission computed tomography (SPECT) may show focal abnormalities that correspond to the neurological deficits. We aim to evaluate the correlation between focal deficit topography and EEG or SPECT abnormalities in 5 new cases. PatientsWe retrospectively reviewed patients attended in a tertiary hospital (January 2010-May 2014) and identified 5 patients (3 men, 2 women) with a mean age of 30.6 ± 7.7 (21-39) years. They presented 3.4 ± 2.6 episodes of headache (range, 2-8) of moderate to severe intensity and transient neurological deficits over a maximum of 5 weeks. Pleocytosis was detected in CSF in all cases (70 to 312 cells/mm3, 96.5-100% lymphocytes) with negative results from aetiological studies. ResultsAt least one EEG was performed in 4 patients and SPECT in 3 patients. Patient 1: 8 episodes; 4 left hemisphere, 3 right hemisphere, and 1 brainstem; 2 EEGs showing left temporal and bilateral temporal slowing; normal SPECT. Patient 2: 2 episodes, left hemisphere and right hemisphere; SPECT showed decreased left temporal blood flow. Patient 3: 3 left hemisphere deficits; EEG with bilateral frontal and temporal slowing. Patient 4: 2 episodes with right parieto-occipital topography and right frontal slowing in EEG. Patient 5: 2 episodes, right hemisphere and left hemisphere, EEG with right temporal slowing; normal SPECT. ConclusionThe neurological deficits accompanying headache in HaNDL demonstrate marked clinical heterogeneity. SPECT abnormalities and most of all EEG abnormalities were not uncommon in our series and they did not always correlate to the topography of focal déficits.

Confusional state as first symptom of HaNDL syndrome.

HaNDL (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) syndrome is an infrequent condition included at group 7 "headache attributed to non-vascular intracranial disorder" in the recent International Classification of Headache Disorders (ICHD-3), code 7.3.5. The description states "migraine-like headache episodes (typically 1-12) accompanied by neurological deficits including hemiparaesthesia, hemiparesis and/or dysphasia, but positive visual symptoms only uncommonly, lasting several hours. There is lymphocytic pleocytosis. The disorder resolves spontaneously within 3 months". In this description confusional state is not considered as a main symptom, even if in the literature this aspect is frequently reported. Here, we report the cases of two young boys presenting with confusional state as the main complaint. The possible pathogenesis of the different clinical presentation is discussed.

Antibodies to DNA repair proteins in headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) patients.

Autoimmune mechanisms have been implicated in the pathogenesis of headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL). Pooled sera of five HaNDL patients and 30 controls (10 multiple sclerosis patients, 10 migraine patients, 10 healthy controls) were screened by protein macroarray. All sera were also individually subjected to immunoprecipitation with neuroblastoma cells and the bound antigens were identified by mass spectrometry. Antibodies to three DNA repair proteins (mitogen-activated protein kinase-4, DNA-dependent protein kinase catalytic subunit, DNA excision repair protein ERCC-6) were identified by both macroarray and immunoprecipitation methods in 3/5 HaNDL sera, but in none of the controls. The presence of DNA repair protein antibodies indicates DNA damage and provides further support for the inflammatory etiology of HaNDL.

Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis Associated with IgM Antibodies to the Epstein-Barr Virus Viral Capsid Antigen

Some authors have suggested that the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) results from an immunological response directed against a viral agent. Here we report a case of HaNDL in an immunocompetent 19-year-old male that could support this hypothesis.

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

Patients with the syndrome of headache with neurological deficits and lymphocytosis (HaNDL) typically present with recurrent and temporary attacks of neurological symptoms and cerebrospinal fluid lymphocytosis. To identify potential HaNDL-associated antibodies directed against neuronal surface and/or synapse antigens, sera of four HaNDL patients and controls were screened with indirect immunohistochemistry, immunofluorescence, cell-based assay, radioimmunoassay, protein macroarray and enzyme-linked immunosorbent assay (ELISA). Although HaNDL sera did not yield antibodies to any of the well-characterized neuronal surface or synapse antigens, protein macroarray and ELISA studies showed high-titer antibodies to a subunit of the T-type voltage-gated calcium channel (VGCC), CACNA1H, in sera of two HaNDL patients. Our results support the notion that ion channel autoimmunity might at least partially contribute to HaNDL pathogenesis and occurrence of neurological symptoms.

Case of syndrome of headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) with focal slowing on electroencephalogram

We describe a case of headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytosis in a patient presenting with a 3-week history of recurrent severe headaches associated with negative sensory symptoms and dysphasia. The patient had no cardiovascular risk factors and no family history of migraines. Neurological examination was unremarkable. Cerebral magnetic resonance imaging was unremarkable. CSF analysis revealed lymphocytosis (leucocytes 84 × 10(6)/L, 100% lymphocytes). Extensive laboratory investigations of CSF and serum did not reveal an infectious, autoimmune or metabolic cause. Visual evoked potentials were normal. Awake electroencephalogram revealed intermittent 3-5 Hz generalised slowing and frontal intermittent rhythmic delta activity, without epileptiform discharges. Repeat CSF analysis showed marked reduction of the total leucocyte count and remained negative for infectious aetiology. Propranolol was commenced, and no recurrence of headache or neurological symptoms was observed at follow-up. An extensive literature review on the topic is discussed.