Facial Sensory Loss Research Articles

Dermatomal Dissociated Facial Sensory Loss due to a Partial Trigeminal Pontine Principal Sensory Nucleus Lesion.

Dermatomal Dissociated Facial Sensory Loss due to a Partial Trigeminal Pontine Principal Sensory Nucleus Lesion.

An In-Depth Analysis of Medullary Strokes at a Tertiary Care Stroke Center: Incidence, Clinical and Radiological Characteristics, Etiology, Treatment, and Prognosis.

Introduction Medullary infarctions (MI) are a rare medical entity that is classified mainly as the more commonly lateral medullary infarcts (LMI) and the less common medial medullary infarcts (MMI). Lateral medullary syndrome, also known as Wallenberg syndrome, results when the medulla oblongata is affected and predominantly occurs secondary to atherosclerotic occlusion of the vertebrobasilar arteries. Previous studies have focused more on the anatomical, clinical, and topographical aspects of medullary infarcts. We describe the incidence of their presentation, radiological findings, etiology, treatment, and outcome at our comprehensive stroke center. Material and method This is a retrospective cohort study of 108 medullary stroke patients with confirmed clinical and radiological diagnoses of MI at Hamad General Hospital, Doha, between January 1, 2018 and December 31, 2020. We evaluated the electronic medical records of all stroke patients. Result During the selected period, a total of 2,912 ischemic strokes were reported. Of these, 843 (28.8%) were posterior circulation strokes. Only 108 (3.7%) patients had medullary strokes. Commonly encountered neurological features were dizziness (94.4%), limb ataxia (84.3%), dysarthria (44.4%), ipsilateral facial sensory loss (32.4%), headache (32.4%), contralateral limb sensory loss (25%), ipsilateral hemiparesis (24%), dysphagia (19.4%), and hiccups (13%). Most strokes reported were either minor (73% with National Institutes of Health Stroke Scale [NIHSS] 1-4) or moderate (26% with NIHSS 5-15). LMIs (87.9%) were the most common, followed by medial paramedian MI (10%). Twenty-five percent had extramedullary involvement, predominantly of the cerebellum (17.6%). Out of the total number of patients, 44 (40.7%) had large vessel atherosclerotic disease, followed by 41 (37.6%) whose stroke was due to small vessel disease, 15 (13.8 %) due to undetermined etiology, and 6 (5.5%) due to arterial dissection. Twenty-eight patients (25.4%) underwent 48-hour Holter monitoring, which detected atrial fibrillation in two patients (1.85%). The majority of patients (98.2%, or 106 patients) received antiplatelet therapy, while 68.5% (74 patients) received single antiplatelet therapy (SAPT), and 29.6% (32 patients) received dual antiplatelet therapy (DAPT). Noteworthy is that only 2.7% (three patients) received thrombolysis as an acute reperfusion therapy. Forty-seven percent (51 patients) were discharged home (mRS 0-2), and 51.9% (56 patients) were transferred to rehabilitation facilities. Follow-up assessments were performed at the stroke clinic for 57.4% (62) of the patients. The assessments found that 46 of the follow-up patients were functionally independent at that time (mRS 0-2). Conclusion This is the first large local study of medullary strokes to determine their frequency, presentation, etiology, treatment, and clinical outcome. Medullary strokes represent 3.7% of total ischemic strokes at our comprehensive stroke center. MI is rare and could present with a variety of neurological and non-specific symptoms that mimic common benign conditions. Prompt and early recognition with a high index of suspicion, the use of posterior NIHSS (POST-NIHSS), and urgent MRI-diffusion-weighted imaging (DWI) of the brain in acute settings can improve early diagnosis and the rate of reperfusion therapy. Further studies are needed to enable the early recognition and treatment of medullary infarcts.

Study of Spectrum of Cavernous Sinus Syndrome in Tertiary Care Centre

ABSTRACT Introduction: Cavernous sinus syndrome (CSS) is a condition with varied etiology. It is characterized by ophthalmoplegia, facial sensory loss, and Horner’s syndrome. The objective of this study was to determine the clinical and radiological presentations and to identify the factors that could help in the identification of etiology. Methods: We prospectively observed all the patients presenting with CSS from October 2019 to September 2021 at a tertiary care hospital. Patients with coronavirus disease 2019 (COVID-19)-associated mucormycosis were excluded. All of these patients underwent detailed clinical examination, hematological, radiological, and specialized investigations based on clinical suspicion. Clinical presentations, imaging characteristics, etiology, and clinical outcomes were analyzed. 45 patients were included in our study. Results: The mean age was 44.6 ± 10.8 years with a female preponderance. The most common presentation was diplopia which was seen in all patients. The commonest cranial nerve involved was the abducens (75.9%). Infections (12 (26.6%)) constituted the commonest cause of CSS, followed by neoplastic involvement in 11 (24.4%), Tolosa–Hunt syndrome in 9 (20%) and vascular involvement in 8 (17.7%) patients. Other inflammatory causes were seen in 5 (11%) patients. Diabetes was positively associated with fungal infections. The presence of bruit was strongly associated with vascular etiology. The presence of sinusitis with bony erosions was strongly associated with fungal infections. Evidence of dilated superior ophthalmic vein with early filling in the angiographic phase was associated with carotid-cavernous fistulas. Conclusions: CSS has variable etiology and radiological presentation. The presence of rapid progressive ophthalmoplegia with underlying diabetes with imaging evidence of sinusitis and bony erosions mandates a high degree of suspicion of fungal infections and empirical antifungal therapy needs to be considered for better outcomes.

Lateral Medullary Syndrome Presenting as Babinski–Nageotte Syndrome with Ipsilateral Upper Motor Neuron Facial Palsy

Babinski–Nageotte syndrome is one of the variants of lateral medullary syndrome (LMS) with contralateral hemiparesis along with classical LMS findings. The simultaneous occurrence of ipsilateral central facial nerve palsy and contralateral hemiparesis is an extremely rare occurrence in LMS. We report the case of a 46-year-old male who presented with complaints of acute onset of vertigo, right-sided (ipsilateral) Horner syndrome, facial sensory loss, upper motor neuron facial nerve palsy, ataxia, and left hemiparesis (contralateral). Magnetic resonance imaging brain suggested acute infarct of the right dorsal medulla and medial aspect of the right cerebellar tonsil with attenuation of the distal segment of the right vertebral artery and right posterior inferior cerebellar artery.

Feasibility and Safety of Cervical Kinematic Magnetic Resonance Imaging in Patients with Cervical Spinal Cord Injury without Fracture and Dislocation.

ObjectiveTo evaluate the feasibility and safety of cervical kinematic MRI (KMRI) in patients with cervical spinal cord injury without fracture and dislocation (CSCIWFD).MethodsThis was a single‐institution case‐only study. Patients with CSCIWFD were enrolled in our institution from February 2015 to July 2019. Cervical radiography and CT were performed first to exclude cervical tumors, and major fracture or dislocation. Then neutral static and kinematic (flexion and extension) MRI was performed for patients who met the inclusion criteria under the supervision of a spinal surgeon. Any adverse events during the KMRI examination were recorded. Patients received surgical or conservative treatment based on the imaging results and patients’ own wishes. The American Spinal Injury Association impairment scale (AIS) grade and the Japanese Orthopedic Association (JOA) score were evaluated on admission, before KMRI examination, and after KMRI examination. For the surgical patients, AIS grade and JOA score were evaluated again 1 week after the operation. The JOA scores were compared among different time points using the paired t‐test.ResultsA total of 16 patients (12 men and 4 women, mean age: 51.1 [30–73] years) with CSCIWFD were included in the present study. Clinical symptoms included facial trauma, neck pain, paraplegia, paresthesia, hyperalgesia, sensory loss or weakness below the injury level, and dyskinesia. On admission, AIS grades were B for 2 cases, C for 5, and D for 9. A total of 14 patients underwent neutral, flexion, and extension cervical MRI examination; 2 patients underwent neutral and flexion examination because they could not maintain the position for a prolonged duration. No patient experienced deterioration of neurological function after the examinations. The AIS grades and JOA scores evaluated post‐examination were similar to those evaluated pre‐examination (P > 0.05) and significantly higher than those on admission (P < 0.05). A total of 12 patients received surgical treatment, 11 of whom underwent anterior cervical discectomy and interbody fusion and 1 underwent posterior C3/4 fusion with lateral mass screws. The remaining 4 patients were offered conservative therapy. None of the patients experienced any complications during the perioperative period. The AIS grade did not change in most surgical patients, except that 1 patient changed from grade C to D 1 week after the operation. The JOA score 1 week after surgery was significantly higher than those on admission and around examination for the surgical patients (P < 0.05).ConclusionCervical KMRI is a safe and useful technique for diagnosis of CSCIWFD, which is superior to static cervical MRI for therapeutic decision‐making in patients with CSCIWFD.

Cavernous Sinus Syndrome

Cavernous sinus syndrome (CSS) is a condition characterized by multiple cranial nerve palsies manifesting with ophthalmoplegia, ptosis, and facial sensory loss due to involvement of adjacent cranial nerves. Tumors, trauma, and vascular, infectious, and noninfectious inflammatory disorders have all been described as causes. Lymphomas have been reported to involve the cavernous sinus, both as primary cavernous sinus lymphomas or as secondary lesions. Here, we describe the case of a 63-year-old-man with untreated chronic lymphocytic leukemia (CLL), diagnosed 4 years earlier, who presented with CSS. Our patient underwent standard chemotherapy, but he succumbed to infection during the neutropenic period.

The clinical significance of persistent trigeminal nerve contrast enhancement in patients who undergo repeat radiosurgery.

OBJECTIVE Contrast enhancement of the retrogasserian trigeminal nerve on MRI scans frequently develops after radiosurgical ablation for the management of medically refractory trigeminal neuralgia (TN). The authors sought to evaluate the clinical significance of this imaging finding in patients who underwent a second radiosurgical procedure for recurrent TN. METHODS During a 22-year period, 360 patients underwent Gamma Knife stereotactic radiosurgery (SRS) as their first surgical procedure for TN at the authors' center. The authors retrospectively analyzed the data from 59 patients (mean age 72 years, range 33-89 years) who underwent repeat SRS for recurrent pain at a median of 30 months (range 6-146 months) after the first SRS. The isocenter was 4 mm, and the median maximum doses for the first and second procedures were 80 Gy and 70 Gy, respectively. A neuroradiologist and a neurosurgeon blinded to the treated side evaluated the presence of nerve contrast enhancement on MRI series at the time of the repeat procedure. The authors correlated the presence of this imaging change with clinical outcomes. Pain outcomes and development of trigeminal sensory dysfunction were evaluated with the Barrow Neurological Institute (BNI) Pain Scale and BNI Numbness Scale, respectively. The mean length of follow-up after the second SRS was 58 months (95% CI 49-68 months). RESULTS At the time of the repeat SRS, contrast enhancement of the trigeminal nerve on MRI scans was observed in 31 patients (53%). Five years after the SRS, patients with this enhancement had lower actuarial rates of complete pain relief after the repeat SRS (27% [95% CI 7%-47%]) than patients without the enhancement (76% [95% CI 58%-94%]) (p < 0.001). At the 5-year follow-up, patients with the contrast enhancement also had a higher risk for trigeminal sensory loss after repeat SRS (75% [95% CI 59%-91%]) than patients without contrast enhancement (26% [95% CI 10%-42%]) (p = 0.001). Dysesthetic pain after repeat SRS was observed for 8 patients with and for 2 patients without contrast enhancement. CONCLUSIONS Trigeminal nerve contrast enhancement on MRI scans observed at the time of a repeat SRS for TN was associated with less satisfactory pain control and more frequently detected facial sensory loss. Residual contrast enhancement at the time of a repeat SRS may warrant consideration of dose reduction or further separation of the radiosurgical targets.

Which Strategy for Petroclival Tumors?

Access to the petroclival region has always been challenging owing to the surrounding neurovascular structures. The ideal approach to the region depends not only on the specific tumor characteristics, but also on surgeon preference. In this video article, we have highlighted the use of the modified Dolenc-Kawase approach to expand the standard anterior petrosectomy corridor for challenging tumors that need additional exposure.1, 2, 3, 4, 5 A 60-year-old woman presented with facial sensory loss and occasional diplopia. On radiological investigation, a densely calcified petroclival lesion was identified (Video 1). A temporal craniotomy with zygomatic osteotomy was performed followed by deroofing of the superior orbital fissure, saucerization of the foramen spinosum, and coagulation and division of the middle meningeal artery. The temporal dura propria was then dissected off the lateral wall of cavernous sinus using a combination of sharp and blunt dissection to expose the modified Dolenc-Kawase rhomboid. A T-shaped dural incision was made along with tentorial sectioning and division of the superior petrosal sinus. Owing to dense calcification in the tumor, a bone Cavitron ultrasonic aspirator (CUSA; Integra LifeSciences, Princeton, New Jersey, USA) was used for debulking of the tumor. Tumor was removed piecemeal alternately using the bone CUSA and scissors. Tumor was dissected from above and below the trigeminal nerve that forms the center of the corridor followed by dissection off the sixth cranial nerve as it enters the Dorello canal. An endoscope may be used at the end to ensure complete removal of the tumor.

Isolated Abducent Nerve Palsy Due to Ipsilateral Pontine Infarction

A 52-year-old man with a history of diabetes mellitus presented with acute horizontal diplopia and transient vertigo for 6 days. Neurologic examination revealed isolated left abducent limitation and right positive Babinski sign with no limbs weakness. The rest of his extraocular movements were normal, and no nystagmus or internuclear ophthalmoplegia was observed. He did not have additional pontomedullary symptoms or signs, such as facial palsy, sensory loss, dysarthria or weakness.

Hemiparesis and Facial Sensory Loss following Cervical Epidural Steroid Injection

Interlaminar cervical epidural steroid injections (ic-ESI) are safe and effective treatment options for the management of acute and chronic radiculopathy, spinal stenosis, and other causes of neck pain not responding to more conservative measures. However, the procedure inherently lends itself to possible spinal cord injury (SCI). Though reports of such events have been documented, the clinical presentation of patients with needle puncture SCI varies. In part, this may be due to anatomic considerations, as symptoms may be dependent on the cervical level intruded, as well as the volume and type of injectate used. Many cases go unreported and therefore the true incidence of cord injections during ic-ESI is not known. Cervical epidurals can be performed by the transforaminal or interlaminar approach. It is generally accepted that ic-ESI is safer than transforaminal epidurals. There are numerous reports of arterial invasion or irritation with the latter despite an inherently greater risk of cord puncture with the former. The likelihood of cord interruption rises when ic-ESIs are performed above C6-C7 as there is a relatively slim epidural layer compared to lower cervical epidural zones. Though most cases of devastating outcomes, such as hemiplegia and death, have been reported during cervical transforaminal epidural injections and rarely with ic-ESI, it is important to understand the symptoms and potential pitfalls of performing any cervical epidural injection. Cervical epidural malpractice claims are uncommon, but exceed those of steroid blocks at all the levels combined, demonstrating the need for improved awareness of potential complications in ic-ESI. Here, we will describe an unusual presentation of a spinal cord injection during an ic-ESI procedure. Key words: Cervical epidural, spinal cord, hemiparesis

Young Patient With Aphasia, Monoparesis, Facial Drop, Facial Sensory Loss, Diplopia, Dysarthria, and Vertical Gaze Palsy

We present a case of a 40-year-old male without previous medical history and medication who denied tobacco smoking or alcohol abuse. He led a healthy lifestyle, and there were no cardiovascular risk factors in his family history. In October 2012, he experienced acute onset of horizontal diplopia while playing with his child. During the transport to the emergency room, he developed a left-sided facial drop and dysarthria. On arrival, he was immediately moved to the computed tomography (CT) room as a candidate for intravenous thrombolysis and was examined by a stroke neurologist. At that time, the patient’s level of consciousness was slightly drowsy, but he was orientated. His pupils were equal with normal reactions to light. He had horizontal diplopia in either direction, more pronounced on the right side. The right eye was nasally deviated. There was no problem with vertical eye movements. He had facial sensory loss on the left side, left-sided facial drop causing dysarthria, and difficulty with word finding. There were no sensorimotor findings in the upper extremities. He was able to raise his right lower extremity, but could not hold it in the upright position for 5 seconds, and the Babinski sign was present on the right side. The National Institutes of Health Stroke Score was 6. With the suspicion of posterior circulation stroke, a noncontrast computed tomographic head scan and CT angiography were ordered. There was no pathology on noncontrast computed tomographic scan. Before CT angiography findings were available, the patient’s symptoms progressed. He became mute but understood verbal commands and developed a vertical gaze palsy. There was confusion …

FOSMN: FACIAL ONSET SENSORY MOTOR NEURONOPATHY. A ‘BENIGN’ DIFFERENTIAL DIAGNOSIS TO BULBAR ONSET MOTOR NEURON DISEASE. THE FIRST COHORT FROM THE UK

IntroductionFacial onset sensory motor neuronopathy (FOSMN) is a rare, slowly progressive bulbar onset motor and sensory neuronopathy. Described only in 2006, it is still under–recognised with fewer than 20 cases...

SENSORY NEURONOPATHY; A CASE REPORT AND A REVIEW OF THE ROLE OF GANGLION NERVE BIOPSY IN DIAGNOSIS

Dorsal root ganglionopathies present with sensory ataxia, areflexia and asymmetrical positive and negative sensory phenomenon of both limb often with trigeminal involvement. There may be associated Adie's pupil1 Despite their...

Facial onset sensory and motor neuronopathy: A neurodegenerative TDP-43 proteinopathy?

Background: Vucic et al. (Brain, 2006) described four adult males with facial sensory loss gradually spreading to cervical and brachial dermatomes associated with LMN weakness of cranio-bulbar and upper extremity (UE) distribution. Autopsy suggested a neurodegenerative process affecting motor neurons and sensory ganglia.

Facial sensory loss in multi-bacillary leprosy patients

Sensation over the face was estimated using the Semmes-Weinstein's monofilament (target force 0-05 gms) in a cohort of multi-bacillary (MB) patients whose clinical and ocular characteristics were available at the time of leprosy diagnosis. Among the 190 MB patients examined, 56 (30%) had areas of sensory impairment somewhere on the face and 43 (23%) had sensory deficit over the lids and/or the malar area. Lagophthalmos (adjusted OR 8.96, 95% CI 0.96-83.50), Type 1 reaction (aOR 2.47, 95% CI 1.11-5-52), history of reactions (aOR 6.36, 95% CI 2.40-16.85) and glove and stocking anaesthesia (aOR 3.49, 95% CI 1-40-8.70) were associated with impaired facial sensation. Hypoesthesia restricted to areas over the lids and/or malar area showed a stronger association with lagophthalmos (aOR 17.5, 95% CI 1.98-154.36). Loss of facial sensation appears to be associated with lagophthalmos in MB patients.

Numbness in the Tip of the Tongue and Lower Lip Caused by Thalamic Hemorrhage

Although patients with isolated oral syndrome or facial sensory loss following stroke of thalamic ventroposteromedial (VPM) nucleus have been reported, there have been no reports of numbness in the tip of the tongue and lower lip. Furthermore, symptoms in the tip of the tongue caused by stroke are typically characterized as gustatory sensory disturbances. A 62-year-old hypertensive man experienced an acute onset of severe numbness in the left tip of the tongue and ipsilateral lower lip. Neurological examination revealed no other abnormalities except for the aforementioned numbness. Head computed tomography showed a small hematoma in the medial part of the right thalamus, most likely within the VPM nucleus. The somatosensory impulse of the tongue is conveyed via the lingual nerve, and it reaches the contralateral medial VPM proper via the trigeminal spinal nucleus. Therefore, thalamic stroke mainly involving the medial VPM proper has the potential to elicit numbness in the tip of the tongue. A major portion of the VPM nucleus is vascularized by the inferolateral arteries. The inferolateral arteries vary greatly in the number and position of the arteries and their tributaries, and small-vessel disease in this territory can present with diverse symptoms because of this complexity. These findings indicate that central neurological involvement should not be overlooked in the case of sensory disturbance restricted to the tip of the tongue and lip.

Gamma Knife thalamotomy for tremor in the magnetic resonance imaging era

The surgical management of disabling tremor has gained renewed vigor with the availability of deep brain stimulation. However, in the face of an aging population of patients with increasing surgical comorbidities, noninvasive approaches for tremor management are needed. The authors' purpose was to study the technique and results of stereotactic radiosurgery performed in the era of MRI targeting. The authors evaluated outcomes in 86 patients (mean age 71 years; number of procedures 88) who underwent a unilateral Gamma Knife thalamotomy (GKT) for tremor during a 15-year period that spanned the era of MRI-based target selection (1996-2011). Symptoms were related to essential tremor in 48 patients (19 age ≥ 80 years and 3 age ≥ 90 years), Parkinson disease in 27 patients (11 age ≥ 80 years [1 patient underwent bilateral procedures]), and multiple sclerosis in 11 patients (1 patient underwent bilateral procedures). A single 4-mm isocenter was used to deliver a maximum dose of 140 Gy to the posterior-inferior region of the nucleus ventralis intermedius. The Fahn-Tolosa-Marin clinical tremor rating scale was used to grade tremor, handwriting, and ability to drink. The median follow-up was 23 months. The mean tremor score was 3.28 ± 0.79 before and 1.81 ± 1.15 after (p < 0.0001) GKT; the mean handwriting score was 2.78 ± 0.82 and 1.62 ± 1.04, respectively (p < 0.0001); and the mean drinking score was 3.14 ± 0.78 and 1.80 ± 1.15, respectively (p < 0.0001). After GKT, 57 patients (66%) showed improvement in all 3 scores, 11 patients (13%) in 2 scores, and 2 patients (2%) in just 1 score. In 16 patients (19%) there was a failure to improve in any score. Two patients developed a temporary contralateral hemiparesis, 1 patient noted dysphagia, and 1 sustained facial sensory loss. Gamma Knife thalamotomy in the MRI era was a safe and effective noninvasive surgical strategy for medically refractory tremor in the elderly or those with contraindications to deep brain stimulation or stereotactic radiofrequency (thermal) thalamotomy.

Opalski’s syndrome: A rare variant of lateral medullary syndrome

Opalski’s syndrome: A rare variant of lateral medullary syndrome

Facial Onset Sensory and Motor Neuronopathy: A Neurodegenerative TDP-43 Proteinopathy? (P03.183)

Objective: We report clinical, electrophysiologic and pathologic features of five new cases of facial onset sensory and motor neuronopathy or FOSMN syndrome. An autopsy performed in one case identified TDP-43 cytoplasmic inclusions. Background Vucic et al. described four adult males in 2006 with facial sensory loss gradually spreading to cervical and brachial dermatomes associated with LMN weakness of cranio-bulbar and upper extremity (UE) distribution. Autopsy in one case suggested a neurodegenerative process affecting motoneurons and sensory ganglia. Design/Methods: A retrospective review of patients referred to Mayo Clinic from 2004 to 2011 identified five males who met the clinical features of FOSMN. Results: The age of onset ranged from 55 to 63 years. The presenting symptoms often years after onset, were facial numbness (5), associated with loss of taste (1), dysphagia (3), and masseter weakness (2). Sensory loss spread to scalp, neck, upper torso and distal UEs. Patients developed facial, masseter, bulbar, neck flexor/extensor and LMN weakness of UEs. Two patients died of respiratory failure 6 and 9 years after onset. Electrodiagnostic studies revealed prolonged to absent trigeminal blink R1 responses, reduced sensory nerve action potentials in UEs, and chronic denervation mainly in cranial and cervical regions. CSF protein elevation (4) ranged from 46 to 96 mg/dL. Brain and cervical MR images were normal. Biopsies of greater auricular and transverse cervical nerves revealed low grade axonal degeneration. A patient aged 65 who died 6 years after onset, came to autopsy. Neuronal loss and gliosis with TDP-43 positive cytoplasmic neuronal and glial inclusions were present in hyploglossal nucleus and cervical motoneurons. Alpha-synuclein positive inclusions were found in substantia nigra, pigmented brainstem nuclei and mesial temporal lobe suggestive of incidental Lewy body disease. Conclusions: FOSMN syndrome joins sporadic ALS, frontotemporal lobar degeneration (FTLD-U) and other neurodegenerative disorders as a TDP-43 proteinopathy. Disclosure: Dr. Bosch has nothing to disclose. Dr. Tracy has nothing to disclose. Dr. Goodman has nothing to disclose. Dr. Dyck has nothing to disclose. Dr. Toro has nothing to disclose. Dr. Giannini has nothing to disclose.

174 Horizontal saccade slowing in a patient with facial onset sensory and motor neuronopathy (FOSMN)

FOSMN is heralded by facial sensory loss with subsequent sensory and motor loss spreading caudally. Few cases have been described in the literature but a single case with upper motor...