Valproate-induced reversible cognitive decline presenting as dementia and associated clinical features: A literature review.

BACKGROUND/AIM: Valproate (VPA) is an effective broad-spectrum anti-seizure medication. Both VPA induced encephalopathy and reversible cognitive decline (VIRCD) have been reported as rare side effects. While the former is well-described in terms of risk factors, mechanism and management, the latter is less recognised and can be easily mistaken for neurodegenerative dementia. In this paper, we present a literature review of VIRCD, describe its clinical features and compare our findings to those in VPA-induced encephalopathy. METHODS: We used PubMed search for valproate induced (dementia OR cognitive impairment OR cognitive decline OR cognitive dysfunction). Patients included were those with normal or well-defined cognitive baseline who presented with dementia after valproate therapy, in whom cognitive decline reversed after VPA dose reduction or discontinuation. Clinical features were compared to published descriptions of VPA-induced encephalopathy. RESULTS: A total of 33 cases in 11 publications were included. Mean age was 51.2 years. Most were being treated for epilepsy on VPA with good seizure control and no encephalopathic features. VPA levels were within the usual quoted range. Mean latency after VPA initiation and symptoms was 6.87 years. Most had parkinsonian features. The most commonly reported cognitive deficits were in short-term memory and processing speed. All recovered fully on VPA discontinuation. CONCLUSION: VIRCD mimics neurodegenerative dementia but is reversible on VPA discontinuation. The absence of encephalopathic features and good seizure control in addition to the prolonged latency make it easy to miss. VIRCD should be considered in relevant patient groups, especially in the presence of extrapyramidal signs.

Severe distinct dysautonomia in RFC1-related disease associated with Parkinsonism.

Biallelic repeat expansions in replication factor C subunit 1 (RFC1) have recently been found to cause cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS). Additional features that have been described include Parkinsonism and a multiple system atrophy (MSA)-like syndrome. CANVAS can include features of dysautonomia, but they are much milder than typically seen in MSA. We report a detailed autonomic phenotype of multisystem RFC1-related disease presenting initially as CANVAS. Our patient presented aged 61 with a sensory ataxic neuropathy who rapidly developed widespread autonomic failure and Parkinsonism. The autonomic profile was of a mixed pre- and post-ganglionic syndrome with progressive involvement of sympathetic and parasympathetic cardiovascular and sudomotor function. The Parkinsonism did not respond to levodopa. We present a patient with CANVAS and biallelic RFC1 expansions who developed Parkinsonism with severe autonomic involvement similar to that seen in classical MSA. The link between MSA and CANVAS remains uncertain.

Pure Autonomic Failure-A Localized Alpha Synucleinopathy with a Potential for Conversion to More Extensive Alpha Synucleinopathies.

Pure autonomic failure (PAF) is an alpha synucleinopathy with predominant involvement of the autonomic ganglia and peripheral nerves. The hallmark clinical feature is orthostatic hypotension. However, genitourinary, sudomotor, and cardiac involvement is also common. Many patients also develop supine hypertension. Almost a quarter of patients can phenoconvert or evolve into Parkinson's disease, multiple system atrophy, and Lewy body dementia in the future. Early severe bladder involvement, higher supine noradrenaline level, early motor involvement, and dream enactment behavior increase the risk of phenoconversion. The diagnosis is confirmed via autonomic function testing and serum noradrenaline measurement. The treatment is mainly supportive. The non-pharmacological treatment includes adequate fluid, dietary salt, compression stockings, and abdominal binders. The drug therapies to improve blood pressure include midodrine, fludrocortisone, pyridostigmine, and droxidopa. The diagnostic criteria need to be updated to incorporate the recent understandings. The treatment of orthostatic hypotension and supine hypertension is mainly based on case series and anecdotal reports. Randomized control trials are needed to ascertain the best treatment strategies for PAF.

Factors Correlated with Unfavorable Outcome after Carpal Tunnel Release Surgery.

OBJECTIVES: Carpal tunnel release surgery has excellent results. The aim of this study was to identify which baseline clinical and demographic factors could predict a good outcome from surgery. Understanding the impact of prognostic factors will enable surgeons to indicate surgical intervention better, provide appropriate preoperative counseling, and manage expectations postoperatively. MATERIALS AND METHODS: A prospective, observational study included 620 carpal tunnel syndrome patients (age 42.38 ± 11.18 years; mean ± standard deviation). After the diagnosis had been confirmed by electrodiagnostic studies, patients underwent open carpal tunnel release surgery. Patients were evaluated initially after 2 weeks and eventually after 6 months. Surgical outcome was compared with presurgical findings. RESULTS: Response to surgery was good in 89.4% and 94.2% after 2 weeks and 6 months, respectively. Factors correlated significantly with unfavorable outcome of surgery included old age, longer duration of symptoms, negative Phalen's test, abnormal two-point discrimination test, and weakness of abductor pollicis brevis muscle. Gender, retrograde radiation, and nocturnal symptoms did not correlate with surgical outcome. CONCLUSION: Elderly patients with longstanding disease, neurological deficits, and negative Phalen's test may not respond to surgery as others. This should be kept in mind in preoperative counseling and postoperative expectations.

LETM presented with causalgia and ensued by sudden death.

BACKGROUND: Longitudinally Extensive Transverse Myelitis LETM is a specific pattern of myelitis wherein at least three continuous vertebral segments are involved. Characteristically, it is a defining feature of neuromyelitis optica NMO. However, it is described in many other etiologies. CASE PRESENTATION: We present a case of 60 year old male who presented with symptoms and signs of regional sympathetic dystrophy RSD followed by symptoms of myelitis. Spinal cord MRI revealed cervical LETM extending to the brainstem. In spite of serological negativity, treatment of suspected neuromyelitis optica spectrum disorder NMOSD was initiated and resulted in symptom relief. Meanwhile, sudden death occurred and autonomic dysreflexia was the main culprit. CONCLUSIONS: This case suggests that RSD could be the mere primary presentation of LETM, discusses the differential diagnoses of LETM in elderly patients, and suggests the possible risk of autonomic dysreflexia in such patients.

Status epilepticus following local anesthesia in a previously healthy adult.

BACKGROUND: Local anesthesia could result in lethal complications if injected in highly vascularized area. Dentist should take care to avoid such complications. CASE PRESENTATION: We present a case of 15 year old girl with a coma following convulsive status epilepticus which developed after inferior alveolar nerve blockade by a dentist. The patient was admitted to the intensive care unit ICU and recovered within several days. CONCLUSION: This case is reported to tell both of dentists and medical staff that although it is uncommon, such complications of local anesthesia should be in mind to be avoided and managed promptly if happened.