Reversible lesion involving the splenium of the corpus callosum caused by phenytoin sodium withdrawal

Wang, Lingling; Wang, Xiaoshan; Shi, Xiangsong; Qiu, Wenchao; Miao, Ailiang

doi:10.1007/s10072-016-2773-9

Cited by 3 publications

(3 citation statements)

References 5 publications

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“…Second, abnormalities of the corpus callosum were impressive, and 26 of 36 cases affected only the splenium. Thus, in addition to seizure, infection, and metabolic disturbance, CMTX1 should be considered as another rare cause of reversible splenial lesion syndrome (RESLES) 68‐70 . Third, the recovery of T2/FLAIR abnormal signals occurred later than clinical symptoms, and even 12 patients did not recover completely during the follow‐up period.…”

Section: Discussionmentioning

confidence: 99%

Systematic review of CMTX1 patients with episodic neurological dysfunction

Tian

Zhao

Zhu

et al. 2020

Ann Clin Transl Neurol

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ObjectiveX‐linked Charcot‐Marie‐Tooth type 1 (CMTX1) is an inherited peripheral neuropathy caused by mutations in the gap junction beta 1 (GJB1) gene, which encodes the connexin32 protein. A small number of patients with GJB1 mutations present with episodic neurological dysfunction and reversible white matter lesions, which has not been adequately reported. Here, we aim to enable clinicians to further understand this particular situation through systematically reviewing all published relevant cases.MethodsWe conducted a comprehensive search of the PubMed electronic database for medical literature relevant to CMTX1 patients with episodic neurological dysfunction and then fully analyzed the general information, clinical manifestations, and characteristics of magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and nerve conduction study (NCS).ResultsWe identified 47 cases of CMTX1 associated with episodic central nervous system (CNS) dysfunction from 38 publications. CMTX1 patients experienced episodic CNS deficits at a young age, ranging from infancy to 26 years, and 45 (95.7%) of them were male. The CNS symptoms manifested as facial, lingual, or limb weakness in 44 (93.6%), dysarthria or dysphagia in 39 (83.0%), facial or limb numbness in 15 (31.9%), and ataxia in 10 (21.3%) patients. The duration of episodic symptoms ranged from 3 minutes to 6 months. Thirty (63.8%) CMTX1 cases have reported obvious predisposing factors, among which the most common factors were infection or fever (27.7%), travel to high altitude (12.8%), and intensive exercise (8.5%). As for brain MRI, most abnormal signals were found in bilateral deep white matter (88.9%) and corpus callosum (80.0%). In addition, most of the NCS results were abnormal, including prolonged latency, reduced amplitude, and slowed conduction velocity. The motor nerve conduction velocity (MNCV) of median nerve was the most detectable and valuable, ranging from 25 to 45 m/s.InterpretationWe have reported the most comprehensive summary of the demographic and clinical profile from 47 CMTX1 patients with episodic CNS deficits and provided new insight into the phenotype spectrum of CMTX1. We hope that our study can help clinicians make early diagnosis and implement the best prevention and treatment strategies for CMTX1 patients with episodic CNS deficits.

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Section: Discussionmentioning

confidence: 99%

Systematic review of CMTX1 patients with episodic neurological dysfunction

Tian

Zhao

Zhu

et al. 2020

Ann Clin Transl Neurol

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“…Antiepileptic drug toxicity and associated changes in salt homeostasis and transhemispheric seizure propagation are other suspected mechanisms (30,31). Cell-cytokine interactions lead to massively elevated extracellular glutamate levels, and this phenomenon is thought to be important in Disturbed consciousness (24,40), somnolence (40)(41)(42), confusion (15,21,40,43), memory problems (15,21,42), disorientated (41,43,44), apathy (40,45), mental abnormalities (46), delirious behavior (24), delirium (47), agraphia (48), ideomotor apraxia (49), alien hand syndrome (49), autotopagnosia (49) Disturbed consciousness (7,9,12,18,(56)(57)(58), lethargy (25), somnolence (25,26,59), confusion (26), cognitive impairment (26), behative impairment (26), behavioral disorders (26), hallucination (25), delirium (25,57), disorientated …”

Section: Discussionmentioning

confidence: 99%

“…Valproate (26), oxcarbazepine (5,33), topiramate (30), levetiracetam (34), phenytoin (3,42), carbamazepine (72) Oxcarbazepine (33) Autoimmune disease…”

Section: Discussionmentioning

confidence: 99%

Reversible Splenial Lesion Syndrome with Some Novel Causes and Clinical Manifestations

Hodes

Zheng

et al. 2020

Intern. Med.

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Objective Reversible splenial lesion syndrome (RESLES) is a clinical radiological syndrome characterized by a reversible lesion of the splenium of the corpus callosum with a decreased apparent diffusion coefficient (ADC) value. The clinical manifestations of RESLES are diverse. Methods Fifteen cases of adult RESLES patients (10 males and 5 females) were retrospectively selected from the radiology system using the key word "corpus callosum" at a university-affiliated tertiary care hospital between May 1, 2015 and December 31, 2019. The possible precipitating factors, clinicoradiological findings and modified Rankin Scale (mRS) on follow-up were then analyzed. Results The patient ages ranged from 22 to 53 years old. The mean age was 34 years old. The most common neurological symptoms included headache (3/15), dizziness (3/15), first onset of seizure (3/15), paroxysmal blurred vision (2/15), vertigo (2/15), amnesia (2/15), and confused consciousness without seizure (2/15), followed by drowsiness (1/15), paresthesia (1/15), dysmetria (1/15) and dysarthria (1/15). The precipitating factors included infection, seizure, anti-epileptic treatment with levetiracetam, carbamazepine, valproate, hyperglycemia, hypoglycemia, cerebral venous sinus thrombosis, and rabies vaccine injection prior to the onset of RESLES. All cases were carefully followed up and had excellent prognoses. Conclusion RESLES manifests as variety of symptoms with less specificity and precipitating factors. Paroxysmal blurred vision may be a relatively specific symptom of RESLES. Levetiracetam, carbamazepine or valproate could be the cause of RESLES, exposure to the rabies vaccine could be another predisposing factors for RESLES as well. RESLES type 1 was therefore found to be highly "reversible" with an excellent prognosis.

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Phenytoin withdrawal

2017

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Reversible lesion involving the splenium of the corpus callosum caused by phenytoin sodium withdrawal

Cited by 3 publications

References 5 publications

Systematic review of CMTX1 patients with episodic neurological dysfunction

Systematic review of CMTX1 patients with episodic neurological dysfunction

Reversible Splenial Lesion Syndrome with Some Novel Causes and Clinical Manifestations

Phenytoin withdrawal

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