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European Journal of Case Reports in... 2024We present a 30-year-old male who sustained a mild traumatic brain injury and then was intubated due to deterioration of consciousness. A head CT scan revealed mild...
UNLABELLED
We present a 30-year-old male who sustained a mild traumatic brain injury and then was intubated due to deterioration of consciousness. A head CT scan revealed mild brain oedema, a fractured nasal bone and mild left thoracic wall haematoma. Despite complete clinical and radiological normalisation within 36 hours, he failed to wean off the ventilator. The patient was found to have subtle bulbar manifestations including dysphonia, dysarthria, and dysphagia, with recurrent left lung collapse. He responded to an empirical pyridostigmine trial despite negative biochemical tests for myasthenia gravis (MG). The patient was weaned successfully from the ventilator, transferred to a long-term care facility, and then discharged home. Classic symptoms and signs of a disease may be absent, but the presence of dysarthria, dysphagia, transient vocal cord palsy, nasal speech, absent gag reflex and respiratory failure in difficult-to-wean patients, with no definitive diagnosis, may warrant an empirical trial of therapy for suspected MG and for the benefit of any doubt.
LEARNING POINTS
"Hidden" cranial injuries may account for subtle bulbar symptoms in victims of traumatic brain injury and should be searched for.Myasthenia gravis has been reported in association with trauma, which comes first and is often difficult to ascertain.A trial of pyridostigmine may be reasonable in difficult-to-wean patients when all other causes have been excluded for the benefit of the doubt.
PubMed: 38584891
DOI: 10.12890/2024_004363 -
Cureus Mar 2024The widespread presence of diabetic peripheral neuropathy (DN) and its related variables among diagnosed diabetes mellitus (DM) patients in Kerala lacks sufficient...
BACKGROUND AND OBJECTIVES
The widespread presence of diabetic peripheral neuropathy (DN) and its related variables among diagnosed diabetes mellitus (DM) patients in Kerala lacks sufficient evidence. The objective of this study was to evaluate the frequency of DN and its related risk variables among those with DM who were receiving care at a tertiary health institution in Kerala.
MATERIALS AND METHODS
This study was conducted in a tertiary health center in Kerala using a cross-sectional design. The diagnosis of diabetes was established by assessing the glycated hemoglobin A1c (HbA1c) level and the fasting blood glucose (FBG) level. A validated survey questionnaire was employed to gather demographic data as well as the medical history of DM and associated ailments. A thorough physical examination, BMI, and blood pressure were recorded. Dermatological, musculoskeletal, neurological, and vascular assessments were conducted. The subjects were assessed for neuropathy using a neuropathy disability score (NDS). Consequently, those who met the criteria for DM were classified into two groups: those with neuropathy and those without neuropathy. All research participants underwent laboratory examinations such as blood lipid profiles, HbA1c, and vitamin B12 complex concentrations.
RESULTS
The study included 200 DM patients; 120 men and 80 women. Study participants were 40-70 years old, with a median age of 53. The prevalence of DN significantly increased with age (p<0.001). The longer a patient had DM, the higher the prevalence of DN, and this variance in percentage was statistically significant (p<0.009) with an OR (odds ratio) of 9.246. A statistically significant 81 (40.5%) of participants graduated, compared to 119 (59.5%) with only higher secondary education or less (OR = 2.042; p = 0.014). Approximately 107 (53.5%) of individuals earned an income under two lakhs, and this disparity was deemed statistically significant (p = 0.003) with an OR of 2.357. In the group of individuals being studied, 53 (26.5%) of them experienced a decrease in pressure sensation, 47 (23.5%) had a decrease or absence of vibration perception, 48 (24%) had an absence or decreased pinprick response, and 46 (23%) had an absent ankle reflex. The study found that the most commonly reported symptoms were tingling (n = 44; 22%), numbness (n = 42; 21%), burning (n = 37; 18.5%), pricking (n = 29; 14.5%), and pain (n = 27; 13.5%). A strong association was found between DN and glycemic status, namely, FBG levels exceeding 140 mg/dL (OR = 4.511, p < 0.001) and HbA1c levels exceeding 6.5% (OR = 3.87, p < 0.001). The prevalence of abnormal vitamin B12 levels in individuals with DN was 63% (p = 0.19), indicating that the finding was not statistically significant. Within the group of individuals with DN being studied, 22% exhibited mild neuropathy, 34% displayed moderate neuropathy, and 44% had severe neuropathy. The DN individuals had significantly reduced ankle dorsiflexion in cases of severe NDS scores compared to those with mild to moderate NDS scores (p = 0.009). During the binary logistic regression analysis, it was shown that the duration of DM (OR = 1.73; p = 0.038) and FBG levels (OR = 2.87; p = 0.018) were determined as predictors for DN.
CONCLUSION
The findings of this study reveal that the duration of diabetes, age, literacy level, income, HbA1c, and FBG were substantially related to a higher likelihood of DN among diabetic patients.
PubMed: 38576639
DOI: 10.7759/cureus.55568 -
Korean Journal of Neurotrauma Mar 2024Since the establishment of Regional Trauma Centers (RTCs) in Korea, significant efforts have been made to improve the quality of care for patients with trauma....
OBJECTIVE
Since the establishment of Regional Trauma Centers (RTCs) in Korea, significant efforts have been made to improve the quality of care for patients with trauma. Simultaneously, the Department of Neurosurgery assigned neurotrauma specialists to RTCs to provide specialized care to patients with traumatic brain injury (TBI). In this study, we sought to determine whether neurotrauma specialists, compared to general neurosurgeons, could make a significant difference in treatment outcomes of patients with TBI.
METHODS
In total, 156 patients with acute TBI who required decompression were included. We reviewed their records and compared the characteristics, outcomes, and prognosis of those who received surgical treatment from either neurotrauma specialists or general neurosurgeons at our institution.
RESULTS
A significant difference was observed between treatment by trauma neurosurgery specialists and general neurosurgeons in time to surgery, with trauma specialists experiencing shorter surgical delays. However, no significant differences existed in mortality rates or Extended Glasgow Outcome Scale scores. Univariate and multivariable regression analyses revealed that lower Glasgow Coma Scale scores, an abnormal pupil reflex, larger transfusion volume, and prolonged time from emergency room admission to surgery were associated with high mortality rates.
CONCLUSION
Neurotrauma specialists can provide prompt surgical treatment to patients with TBI compared to general neurosurgeons. Our study did not reveal a significant difference in outcomes between the two groups. However, it is clear that rapid decompression is effective in patients with impending brain herniation. Therefore, the effectiveness of neurotrauma specialists needs to be confirmed through further systematic studies.
PubMed: 38576504
DOI: 10.13004/kjnt.2024.20.e12 -
Journal of Orthopaedic Case Reports Mar 2024Numerous reflexive responses have been documented as alterations to the Babinski sign within upper motor neuron lesions. However, scant attention has been given to...
INTRODUCTION
Numerous reflexive responses have been documented as alterations to the Babinski sign within upper motor neuron lesions. However, scant attention has been given to reflexes beyond these, which exhibit independence from the extensor plantar response. These reflexes predominantly form polysynaptic arcs, with nociceptive stimuli acting as afferents.
CASE REPORT
The reflex was serendipitously discovered in an 18-year-old female patient who presented with spastic paraplegia with bowel and bladder involvement, as a consequence of an aneurysmal bone cyst of the D3 (dorsal) vertebrae, and the same was named after the authors as "Yadav-Kunal reflex" which can be defined as: "In individuals with spastic paraparesis, forcibly plantarflexing the toes will result in sudden jerky flexion of the knee and hip on the same side." This novel reflex was further investigated and validated in two additional patients with spastic paraplegia: one, a 45-year-old female with D9-D10 Pott's spine and bowel and bladder involvement, and the other, a 65-year-old male with D10-D11 compressive myelopathy and bowel and bladder involvement. This reflex was meticulously tracked until the abatement of spasticity following surgical intervention. Notably, its manifestation was evident in individuals experiencing spastic paraparesis, dissipating concomitantly with the resolution of spasticity - a direct clinical correlation. Conversely, the reflex was conspicuously absent in cases of flaccid paraplegia.
CONCLUSION
Spasticity, characterized by an increase in muscle tone on swift stretching movements, is a manifestation of a stretch reflex disorder. This condition is primarily induced by lesions affecting upper motor neurons. The activation of muscle spindles in toe dorsiflexors (primarily governed by the L5 nerve) occurs during forceful elongation caused by plantarflexion.
PubMed: 38560301
DOI: 10.13107/jocr.2024.v14.i03.4326 -
Frontiers in Neurology 2024The suppression head impulse paradigm (SHIMP) involves suppression of the vestibulo-ocular reflex (VOR) and anticompensatory saccades generated thereof. SHIMP is gaining...
The suppression head impulse paradigm (SHIMP) involves suppression of the vestibulo-ocular reflex (VOR) and anticompensatory saccades generated thereof. SHIMP is gaining importance to understand vestibular compensation with its different parameters (VOR gain/peak saccadic velocity PSV/latency of saccades). SHIMP studies are emerging in adults, but pediatric studies have hardly been performed. This study is a retrospective case note audit over a period of 2 months in a tertiary pediatric vestibular center in the United Kingdom to investigate whether SHIMP is safe/robust to be used in children conforming to existing standards/norms in normal children and whether it yields any meaningful inferences in pediatric vestibular hypofunction. This is the largest pediatric SHIMP study to date. A total of 44 referred children (6-18 years, female children>male children) with a range of complaints from dizziness, imbalance, motor incoordination, postural instability, and hearing loss were included, and their SHIMP parameters were measured. All children underwent comprehensive functional/objective audiovestibular assessments. Two groups were defined-Group A with normal vestibular function and Group B with abnormal vestibular function. The normal population showed an average SHIMP VOR gain of 0.98+/-0.08 and latency of overt saccades at 215.68+/-46.16 milliseconds agreeing with published evidence. The PSV of overt saccades was 315.39+/-56.3/s, and there was a gain asymmetry of 7.42+/-4.68 between the sides. Statistically significant differences with moderate/large effect sizes were observed between the groups in terms of VOR gain and PSV but not in saccade latencies. Covert saccades were rare in SHIMP, while overt saccades were observed in 100% of children. VOR gain difference between the head impulse paradigm (HIMP) and the SHIMP was significant as well. We observed statistically significant differences in side asymmetry of VOR gain between the groups. Furthermore, we identified a group of children with cerebellar lesions where overt saccades in SHIMP were rather low in number. Further research is recommended to investigate pediatric PSV, asymmetry, and inability to generate overt saccades that may suggest useful means to assess compensation and central function. We conclude that SHIMP yields valuable information and is a safe, easy to perform, and a reliable test that should be used in children to supplement HIMP.
PubMed: 38550340
DOI: 10.3389/fneur.2024.1297707 -
Frontiers in Veterinary Science 2024A 2.5-year-old female entire Pomeranian dog was presented for acute paraparesis progressing within 2 days to paraplegia. General physical examination was unremarkable....
A 2.5-year-old female entire Pomeranian dog was presented for acute paraparesis progressing within 2 days to paraplegia. General physical examination was unremarkable. Neurological examination showed paraplegia without nociception, a mass reflex upon testing perineal reflexes and withdrawal reflexes in the pelvic limbs and patellar hyperreflexia. Cutaneous trunci reflexes were absent caudal to the level of the 6th thoracic vertebra. Spinal hyperesthesia was present. Neuroanatomical localization was consistent with a T3-L3 myelopathy. Hematological and biochemical blood tests [including C-reactive protein (CRP)] were within reference ranges. MRI of the spinal cord from the level of the 1st thoracic vertebra to the sacrum revealed a patchy, ill-defined, moderate to marked T2W hyperintense, contrast enhancing intramedullary lesion extending from T1 to L4. Medical treatment based on a working diagnosis of meningomyelitis of unknown cause was initiated with corticosteroids and methadone based on pain scores. Prognosis was grave and after 3 days without return of nociception, the dog was euthanized according to the owners' wishes. Post-mortem histopathological examination of the brain and spinal cord yielded a morphological diagnosis of severe, segmental, bilateral and fairly symmetrical, necrotizing lymphohistiocytic leukomyelitis, with a non-suppurative angiocentric leptomeningitis. Some minor, focal, lymphocytic perivascular cuffing was found in the medulla oblongata as well, but otherwise there were no signs of brain involvement. No infectious causes were identified with ancillary tests. This case report underlines the importance of including meningomyelitis in the differential diagnosis list of dogs presented for acute progressive neurological signs referable to a myelopathy.
PubMed: 38545560
DOI: 10.3389/fvets.2024.1303084 -
Journal of the Neurological Sciences Apr 2024The epidemiology and etiology of facial onset sensory and motor neuronopathy (FOSMN), a rare syndrome that initiates with facial sensory disturbances followed by bulbar...
The epidemiology and etiology of facial onset sensory and motor neuronopathy (FOSMN), a rare syndrome that initiates with facial sensory disturbances followed by bulbar symptoms, remain unknown. To estimate the prevalence of FOSMN in Japan and establish the characteristics of this disease, we conducted a nationwide epidemiological survey. In the primary survey, we received answers from 604 facilities (49.8%), leading to an estimated number of 35.8 (95% confidential interval: 21.5-50.2) FOSMN cases in Japan. The secondary survey collected detailed clinical and laboratory data from 21 cases. Decreased or absent corneal and pharyngeal reflexes were present in over 85% of the cases. Electrophysiological analyses detected blink reflex test abnormalities in 94.1% of the examined cases. Immunotherapy was administered in 81% of cases and all patients received intravenous immunoglobulin. Among them, 35.3% were judged to have temporary beneficial effects evaluated by the physicians in charge. Immunotherapy tended to be effective in the early stage of disease. The spreading pattern of motor and sensory symptoms differed between cases and the characteristics of the motor-dominant and sensory-dominant cases were distinct. Cases with motor-dominant progression appeared to mimic amyotrophic lateral sclerosis. This is the first nationwide epidemiological survey of FOSMN in Japan. The clinical course of FOSMN is highly variable and motor-dominant cases developed a more severe condition than other types of cases. Because clinical interventions tend to be effective in the early phase of the disease, an early diagnosis is desirable.
Topics: Humans; Japan; Amyotrophic Lateral Sclerosis; Neurologic Examination; Face
PubMed: 38520939
DOI: 10.1016/j.jns.2024.122957 -
Human Molecular Genetics Jun 2024We have generated using CRISPR/Cas9 technology a partially humanized mouse model of the neurometabolic disease phenylketonuria (PKU), carrying the highly prevalent PAH...
We have generated using CRISPR/Cas9 technology a partially humanized mouse model of the neurometabolic disease phenylketonuria (PKU), carrying the highly prevalent PAH variant c.1066-11G>A. This variant creates an alternative 3' splice site, leading to the inclusion of 9 nucleotides coding for 3 extra amino acids between Q355 and Y356 of the protein. Homozygous Pah c.1066-11A mice, with a partially humanized intron 10 sequence with the variant, accurately recapitulate the splicing defect and present almost undetectable hepatic PAH activity. They exhibit fur hypopigmentation, lower brain and body weight and reduced survival. Blood and brain phenylalanine levels are elevated, along with decreased tyrosine, tryptophan and monoamine neurotransmitter levels. They present behavioral deficits, mainly hypoactivity and diminished social interaction, locomotor deficiencies and an abnormal hind-limb clasping reflex. Changes in the morphology of glial cells, increased GFAP and Iba1 staining signals and decreased myelinization are observed. Hepatic tissue exhibits nearly absent PAH protein, reduced levels of chaperones DNAJC12 and HSP70 and increased autophagy markers LAMP1 and LC3BII, suggesting possible coaggregation of mutant PAH with chaperones and subsequent autophagy processing. This PKU mouse model with a prevalent human variant represents a useful tool for pathophysiology research and for novel therapies development.
Topics: Animals; Mice; Phenylketonurias; Humans; Disease Models, Animal; Phenylalanine Hydroxylase; Brain; CRISPR-Cas Systems; Autophagy; Mutation; Liver
PubMed: 38520741
DOI: 10.1093/hmg/ddae051 -
Cardiovascular Diabetology Mar 2024There is a paucity of contemporary data on the prevalence and prognostic significance of cardiac autonomic neuropathy (CAN) from community-based cohorts with type 2... (Observational Study)
Observational Study
BACKGROUND
There is a paucity of contemporary data on the prevalence and prognostic significance of cardiac autonomic neuropathy (CAN) from community-based cohorts with type 2 diabetes assessed using gold standard methods. The aim of this study was to assess these aspects of CAN in the longitudinal observational Fremantle Diabetes Study Phase II (FDS2).
METHODS
FDS2 participants were screened at baseline using standardised cardiovascular reflex tests (CARTs) of heart rate variation during deep breathing, Valsalva manoeuvre and standing. CAN (no/possible/definite) was assessed from the number of abnormal CARTs. Multinomial regression identified independent associates of CAN status. Cox proportional hazards modelling determined independent baseline predictors of incident heart failure (HF) and ischaemic heart disease (IHD), and all-cause mortality.
RESULTS
Of 1254 participants assessed for CAN, 86 (6.9%) were outside CART age reference ranges and valid CART data were unavailable for 338 (27.0%). Of the remaining 830 (mean age 62.3 years, 55.3% males, median diabetes duration 7.3 years), 51.0%, 33.7% and 15.3% had no, possible or definite CAN, respectively. Independent associates of definite CAN (longer diabetes duration, higher body mass index and resting pulse rate, antidepressant and antihypertensive therapies, albuminuria, distal sensory polyneuropathy, prior HF) were consistent with those reported previously. In Kaplan-Meier analysis, definite CAN was associated with a lower likelihood of incident IHD and HF versus no/possible CAN (P < 0.001) and there was a graded increase in all-cause mortality risk from no CAN to possible and definite CAN (P < 0.001). When CAN category was added to the most parsimonious models, it was not a significant independent predictor of IHD (P ≥ 0.851) or HF (P ≥ 0.342). Possible CAN (hazard ratio (95% CI) 1.47 (1.01, 2.14), P = 0.046) and definite CAN (2.42 (1.60, 3.67), P < 0.001) increased the risk of all-cause mortality versus no CAN.
CONCLUSIONS
Routine screening for CAN in type 2 diabetes has limited clinical but some prognostic value.
Topics: Male; Humans; Middle Aged; Female; Diabetes Mellitus, Type 2; Prognosis; Prevalence; Heart; Cardiovascular System; Coronary Artery Disease; Heart Failure
PubMed: 38500197
DOI: 10.1186/s12933-024-02185-3 -
Experimental Neurology Jun 2024Spasticity is a complex and multidimensional disorder that impacts nearly 75% of individuals with spinal cord injury (SCI) and currently lacks adequate treatment...
Spasticity is a complex and multidimensional disorder that impacts nearly 75% of individuals with spinal cord injury (SCI) and currently lacks adequate treatment options. This sensorimotor condition is burdensome as hyperexcitability of reflex pathways result in exacerbated reflex responses, co-contractions of antagonistic muscles, and involuntary movements. Transcutaneous spinal cord stimulation (tSCS) has become a popular tool in the human SCI research field. The likeliness for this intervention to be successful as a noninvasive anti-spastic therapy after SCI is suggested by a mild and transitory improvement in spastic symptoms following a single stimulation session, but it remains to be determined if repeated tSCS over the course of weeks can produce more profound effects. Despite its popularity, the neuroplasticity induced by tSCS also remains widely unexplored, particularly due to the lack of suitable animal models to investigate this intervention. Thus, the basis of this work was to use tSCS over multiple sessions (multi-session tSCS) in a rat model to target spasticity after SCI and identify the long-term physiological improvements and anatomical neuroplasticity occurring in the spinal cord. Here, we show that multi-session tSCS in rats with an incomplete (severe T9 contusion) SCI (1) decreases hyperreflexia, (2) increases the low frequency-dependent modulation of the H-reflex, (3) prevents potassium-chloride cotransporter isoform 2 (KCC2) membrane downregulation in lumbar motoneurons, and (4) generally augments motor output, i.e., EMG amplitude in response to single pulses of tSCS, particularly in extensor muscles. Together, this work displays that multi-session tSCS can target and diminish spasticity after SCI as an alternative to pharmacological interventions and begins to highlight the underlying neuroplasticity contributing to its success in improving functional recovery.
Topics: Animals; Spinal Cord Injuries; Rats; Homeostasis; Reflex, Abnormal; Rats, Sprague-Dawley; Spinal Cord Stimulation; Female; Chlorides; Muscle Spasticity; Neuronal Plasticity
PubMed: 38493983
DOI: 10.1016/j.expneurol.2024.114754