OBJECTIVE: Limited data provides guidance on the management of vestibular schwannomas (VSs) that have progressed despite primary Gamma Knife radiosurgery (GKRS). The present article reports our long-term experience after repeat GKRS for VS with sustained progression after solely primary GKRS management.
METHODS: A retrospective review of 1997 patients managed between 1987 and 2023 was conducted. Eighteen patients had sustained tumor progression after primary GKRS and underwent repeat GKRS. The median repeat GKRS margin dose was 11 Gy (IQR: 11-12), the median tumor volume was 2.0 cc (IQR: 1.3-6.3), and the median cochlear dose in patients with preserved hearing was 3.9 Gy (IQR: 3-4.1). The median time between initial and repeat GKRS was 65 months (IQR: 38-118).
RESULTS: The median follow-up was 70 months (IQR: 23-101). After repeat GKRS, two patients had further tumor progression at 4 and 21 months and required partial resection of their tumors. The 10-year actuarial tumor control rate after repeat GKRS was 88%. Facial nerve function was preserved in 13 patients who had House-Brackmann grade 1 or 2 function at the time of repeat GKRS. Two patients with serviceable hearing preservation (Gardner-Robertson grade 1 or 2) at repeat GKRS retained that function afterwards. In patients with tinnitus, vestibular dysfunction, and trigeminal neuropathy, symptoms remained stable or improved for 16/16 patients, 12/15 patients, and 10/12 patients, respectively. One patient developed facial twitching in the absence of tumor growth 21 months after repeat GKRS.
CONCLUSIONS: Repeat GKRS effectively controlled tumor growth and preserved cranial nerve outcomes in most patients whose VS had sustained progression after initial primary radiosurgery.

We reported a 61-year-old man presented with 10-month progressing left sciatic neuropathy and 10-day right facial neuropathy. Serum amphiphysin-IgG was positive. 18F-FDG PET/CT of the whole body showed no signs of malignancy. Treatment with plasma exchange and oral prednisone relieved the symptoms. Nine months later, right hemiparesis and seizure of right limbs developed. 18F-FDG and 18F-PBR06 (18 kDa translocator protein, TSPO) radioligand PET/MRI of the whole body revealed intense uptake in the intracranial lesions. Intracranial lymphoma was diagnosed by stereotactic needle brain biopsy. Mononeuropathies could be paraneoplastic syndromes. TSPO shows high uptake in intracranial lymphoma on 18F-PBR06 PET images.

OBJECTIVE: To analyze the diagnosis, treatment and rehabiltation of patients with marginal mandibular branch of the facial nerve (MMB).
METHODS: We have collected 6 patients (mean age 40 [33.8; 44] years) with isolated lesion of MMB that innervates the depressor labii inferioris and chin muscle. The illness duration without any improvement was 35 [13; 44] days. Diagnosis and treatment were carried out according to the special algorithm developed and implemented at the N.V. Sklifosovsky Research Institute of Emergency Medicine.
RESULTS: With needle myography of the muscle that lowers the lower lip, the change in the ratio of the maximum amplitudes of the interference pattern (MAIP) in all patients exceeded 15%, and in 2 cases it was more than 90%. Comparing with the healthy face side, a change of the MAIP ratio less than 90% was considered as the biomarker of favorable prognosis, with conservative treatment recommendations, e.g. the set of exercises with targeted effects on depressor labii inferioris. With regular exercises, patients noted positive dynamics of restoring the symmetry of the smile in 1-2 months of the disease, full recovery - in 4-5 months. In case of exercises rejection, there was no positive dynamics. A change in the MAIP ratio more than 90% or the absence of motor unit potentials was considered as the biomarker of an unfavorable outcome and an indication for surgical treatment. After surgical treatment, the improvement occurred within 4-5 months. In conservative treatment group, there were no positive changes even with regular exercises.
CONCLUSIONS: The diagnosis of an isolated lesion of MMB is established clinically using a protocol of step-by-step assessment of facial muscle function, and tactics is determined by needle myography with depressor labii inferioris. Even with favorable myographic predictors, spontaneous recovery may not occur, exercises with a targeted effect on the depressor labii inferioris are required, and in the presence of unfavorable predictors, surgical treatment is reccomended.
UNASSIGNED: Провести анализ диагностики, лечения и реабилитации группы пациентов с изолированным поражением краевой нижнечелюстной ветви лицевого нерва (КНВ ЛН).
UNASSIGNED: Выполнено обследование 6 пациентов в возрасте 40 [33,8; 44] лет с изолированным поражением КНВ ЛН, иннервирующей мышцу, опускающую нижнюю губу и подбородочную мышцу, разной этиологии, давностью 35 [13; 44] дней, без предшествующей положительной динамики. Диагностику и лечение проводили по алгоритму, разработанному и внедренному в НИИ СП им. Н.В. Склифосовского.
UNASSIGNED: При игольчатой электромиографии мышцы, опускающей нижнюю губу, изменение отношений максимальных амплитуд интерференционного паттерна (МАИП) у всех пациентов превышало 15%, причем в 2 случаях оно было >90%. Изменение отношения МАИП <90% в сравнении со здоровой стороной считали биомаркером благоприятного прогноза и рекомендовали консервативное лечение — комплекс упражнений с прицельным воздействием на мышцу, опускающую нижнюю губу. При регулярных занятиях пациенты отмечали положительную динамику восстановления симметричности улыбки через 1—2 мес, полное восстановление через 4—5 мес, в случае отказа от занятий положительная динамика отсутствовала. Изменение отношения МАИП >90% или отсутствие потенциалов двигательных единиц считали биомаркером неблагоприятного прогноза и показанием к оперативному лечению. После хирургического лечения улучшение отмечено через 4—5 мес, а без хирургического лечения даже при регулярных занятиях положительной динамики не было.
UNASSIGNED: Диагноз изолированного поражения КНВ ЛН устанавливают клинически с использованием протокола пошаговой оценки функции мышц лица, а тактику определяют посредством игольчатой миографии мышцы, опускающей нижнюю губу. Даже при благоприятных электромиографических предикторах спонтанное восстановление может не наступить, требуется выполнение упражнений с прицельным воздействием на мышцу, опускающую нижнюю губу, а при наличии неблагоприятных предикторов показано оперативное лечение.

BACKGROUND: Facial nerve palsy is a cardinal manifestation of neurosarcoidosis, but dedicated studies of this disease feature have not been conducted. We sought to clarify the impact of facial palsy on the diagnosis of neurosarcoidosis, its subsequent clinicoradiographic evolution, and eventual treatment decisions.
METHODS: A single-center retrospective analysis of patients with neurosarcoidosis and facial palsy was conducted over the preceding 10 years (01/01/2011-08/12/2021).
RESULTS: 23/218 (10.6%) patients with neurosarcoidosis developed facial neuropathy. It was the inaugural manifestation of neurosarcoidosis in 17/23 (73.9%) and presented in isolation of other neurologic deficits or extra-facial MRI abnormalities in 12/23 (52.2%). At onset, facial palsy was unilateral in 20/23 (87.0%), and multiple cranial neuropathies were seen in 8/23 (34.8%). Non-facial inflammatory MRI abnormalities were observed in 6/15 (40.0%) patients at onset with leptomeningitis being most common (5/15, 33.3%). 13/23 (56.5%) experienced a second attack of neurosarcoidosis at a median of 8 months, including 3/23 (13.0%) with recurrent facial palsies. In the 12 patients with isolated facial paresis at onset, 4/12 (33.3%) remained free of new deficits or neuroimaging abnormalities by last follow-up. 17/23 (73.9%) eventually required initiation of steroid-sparing immunosuppressants, almost all for development of non-facial disease. The final median House-Brackmann score was 1.
CONCLUSIONS: Facial neuropathy occurred less commonly than historically reported, and it often acts as a forerunner to systemic sarcoidosis and more widespread neurologic disease. Recurrent attacks of neurosarcoidosis occur early at high frequency following facial palsy. Recovery of facial nerve function is typically excellent.

Objective  Vestibular schwannoma (VS) treated with Gamma Knife stereotactic radiosurgery (SRS) was typically performed at 50% isodose line (IDL50); however, the impact of IDL variation on outcomes is poorly understood. This study aimed to compare tumor control (TC) and toxicities between treatment at 40% (IDL40) and 50% (IDL50). Methods  Sporadic/unilateral VS patients treated with SRS dose 12 to 14 Gy and prescription isodose volume ≤10cm 3 were included. Propensity score matching was applied to IDL40 cohort to generate an IDL50 companion cohort, adjusting for age and prescription isodose volume. After exclusion of patients with follow-up <24 months, there were 30 and 28 patients in IDL40 and IDL50 cohorts, respectively. Results  Median follow-up time was 96 months (24-225 months). Actuarial and radiographic TC rates were 91.8% and clinical TC was 96.2% both at 5 and 10 years. TC was higher in IDL40 cohort but not significant (96.4 vs. 86.7%; p  = 0.243). Hearing preservation (HP) rates were 71.9 and 39.2% at 5- and 10-year intervals, with significantly higher rates of HP noted in IDL40 cohort (83.3 vs. 57.1% at 5-year interval; 62.5 vs. 11.4% at 10-year interval; p  = 0.017). Permanent facial neuropathy occurred in two patients, both from the IDL50 cohort (3.5%). Rates of post-SRS steroid treatment or shunt placement for hydrocephalus were slightly higher in IDL50 patients (6.9 vs. 17.9%; p  = 0.208 and 3.3 vs. 7.1%; p  = 0.532). Conclusion  For treatment of VS with SRS, dose prescription at IDL40 or IDL50 provides excellent long-term TC and toxicity profiles. IDL40 may be associated with improved long-term HP.

BACKGROUND: Cranial nerve involvement is not commonly encountered in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); this is especially true for involvement of the hypoglossal nerve. Neither Beevor\'s sign nor its inverted form has previously been described in CIDP.
METHODS: A 28-year-old man presented with distal-predominant limb weakness and numbness at the age of 18. A diagnosis of CIDP was made, which was confirmed by electrodiagnostic evidence of demyelination. He responded well to intravenous immunoglobulin and glucocorticoid treatment and achieved remission for 5 years. However, the same symptoms relapsed at the age of 28 and lasted for 10 months. On examination, in addition to limb sensory impairment and muscle weakness, mild bilateral facial paresis, tongue atrophy and fasciculations, and inverted Beevor\'s sign were also observed. A brief literature review of cranial nerve involvements in CIDP and Beevor\'s sign or its inverted form were also performed.
CONCLUSIONS: Cranial nerves may be affected in patients with CIDP. Facial palsy is most frequently present, while hypoglossal nerve involvement is rare. Inverted Beevor\'s sign can appear in CIDP patients.

OBJECTIVE: To describe and compare the cumulative incidence and clinical progression of ocular neuropathies in diabetic dogs vs nondiabetic dogs following cataract surgery.
METHODS: Medical records of 196 diabetic and 442 nondiabetic dogs who underwent cataract surgery between 2004 and 2015 were reviewed. The percentage of patients affected by neuropathy and potential risk factors were compared between groups.
RESULTS: Patients with diabetes mellitus (DM) were 20.4 times more likely to develop an ocular neuropathy than patients without DM (12.24% vs 0.68%). Twenty-four diabetic patients were affected by mononeuropathies or polyneuropathies including Horner\'s syndrome (n = 20), facial neuropathy (n = 5), and neurogenic keratoconjunctivitis sicca (NKCS) (n = 5). The odds of a diabetic patient developing Horner\'s syndrome and NKCS were 86.3 and 20.7 times higher than a nondiabetic patient, respectively. The average duration of DM prior to diagnosis of neuropathy was 659 days (range 110-2390 days; median 559 days). Complete resolution was achieved in 10 of 22 neuropathies (45%) within an average of 248 days (range 21-638 days; median 187 days) after diagnosis.
CONCLUSIONS: The odds of developing an ocular neuropathy, specifically Horner\'s syndrome and NKCS, are statistically higher in diabetic patients compared to nondiabetic patients. Neuropathies were observed as a long-term complication in this group of diabetic patients, and complete resolution of the neuropathy was observed in less than half of the affected population.

Perineural invasion is a targeted cellular proliferation guided by neurotrophins, rather than a simple diffusion of tumor in a path of least resistance. Invasion of cranial nerves by squamous cell carcinoma can represent an important diagnostic dilemma. It commonly presents as a distinct clinical neurological syndrome of combined isolated trigeminal and facial neuropathies. The focal cancer source may have been overlooked or remain occult. This case series illustrates diverse clinical presentations and neuroimaging challenges in four patients with squamous cell carcinoma of the cranial nerves. Anatomical pathways linking the trigeminal and facial nerves are reviewed, with emphasis on the auriculotemporal and pterygopalatine nerves. A successful neuroimaging strategy requires a targeted multimodality analysis of specific anatomical loci at the base of the skull. Attention must be directed to subtle radiological findings, such as obliteration of fat planes and linear enhancement along nerve branches, rather than bulky tumor tissue or bony invasion. Despite advances in microsurgical dissection and targeted radiotherapy, recovery of established neuropathic deficits is not expected. The prognosis remains poor in cases of advanced disease, emphasizing the importance early diagnosis by clinical acumen and focused neuroimaging.