Moyamoya Disease

Moyamoya Disease

Definition

Idiopathic chronic intracranial vascular steno-occlusive disease characterized by progressive stenosis of the terminal segments of the internal carotid arteries bilaterally and/or partial stenosis or occlusion of the proximal portions of the anterior cerebral arteries and the middle cerebral arteries and formation of an abnormal network of vessels at the base of the skull

Causes
- idiopathic
- SCD association

Findings
Classification
- Suzuki

Puff of smoke sign


Facial Nerve Anatomy

Facial nerve anatomy

Supranuclear pathways
Somatomotor cortex: Broadmann area 4, 6, 8
Volitional component: bilateral cortico-nuclear tract innervation
Emotional component: basal ganglia and limbic system control
Other inputs: visual system (involved in blink reflex), trigeminal nerve and nuclei (involved in corneal reflex), auditory nuclei (involuntary closure of eye in response to loud noise)
- wrinkling forehead: frontal branch components of facial nucleus receives innervation from the cortex of both the sides
- involuntary vs voluntary smile are different paths

Course
Pontomedullary junction → Posterior cranial fossa → Internal acoustic meatus → Fundus of Internal acoustic meatus  → Bony facial canal  → Stylomastoid foramen

- nucleus: motor, salivatory, and solitary
- IAC: 7up, coke down
- 5 peripheral branches: Two Zombies Burggered My Cat
Upper temporofacial division gives: Temporal and Zygomatic
Lower cervicofacial division gives: Buccal, Marginal mandibula, Cervical

Facial Nerve Subdivision and Functions
Branchial motor: Muscles of facial expression, Posterior belly of digastric muscle, Stylohyoid muscle, Stapedius muscle
Visceral motor: Salivation (lacrimal, submandibular, and sublingual), Nasal mucosa or mucous membrane
General sensory: Sensory to auricular concha, External auditory canal, Tympanic membrane
Special sensory Chorda tympani nerve (taste to anterior 2/3 of the tongue)

Donor nerve
The most common donor nerve for facial nerve grafting is sural and greater auricular nerves.

White matter change

White matter change


Fazekas scores

Deep White Matter Fazekas
1 → “1-hit wonder”
Punctate foci — just tiny spots, like little dots.
2 → “2 = two-gether”
Beginning confluence of foci — two dots coming together, mild merging.
3 → “3 is a crowd”
Large, confluent areas — everything blending into a mess, too much white.

Periventricular Fazekas
1 → “1 is a pencil”
✏️ Pencil looks a number 1 (pencil-thin “cap” or rim along ventricle).
2 → “2 curves into a halo”
⭕ Smooth halo of hyperintensity — picture the roundness of 2 encircling the ventricle.
3 → “3 branches out”
🌿 Irregular fingers of white matter reaching into the deep — the edge of the number 3 look like irregular finger

Multiple Sclerosis - DMT

Multiple Sclerosis - DMT

Consider
- risk/ benefit ratio
- patient features: age; disease duration; lesion burden; JCV; comorbidities; tolerance; pregnancy
- dosing: injectable; oral; infusion
- approach: eslation (safety) vs intensive (risks)

Efficacy
- modest (up to 34% red): glatiramer, interferon, teriflunomide
- moderate (up to 58% red): DMT, S1P modulator, cladribine
- high (up to 68% red): alemtuzumabm, natalizumab, anti-CD20

Modest
Glatiramer (Copaxone)
- MHC binding
- 6 months for effect
- SQ 40 mg 3x/wk or 20 mg/d
- no monitoring blood work

Interferon
INF- beta 1a (Avonex, Rebif); INF- beta 1b (Extavia, Betaseron); INF - beta 1a (Plegridy)
- inh cytokines
- flu-like symptoms and worse spasticity
- monitor: CBC w/ diff & LFT 1-3-6, then q3 mo; TSH q6 mo

Teriflunomide (Aubagio)
- inh DHO dehydrogenase
- 7 or 14 mg PO daily
- alopecia, diarrhea, and inc LFTs
- pretest: TB, pregnancy, LFTs
- monitor: CBC w/ diff and LFTs q6; if LFTs > 3x abnormal -> cholestyramine

Moderate
Fumarates
Dimethyl (Tecfidera); Diroximel (Vumerity)
- activates Nrf2
- 120 mg BID for 7d, and then 240 mg BID; 231 mg BID for 7d, and then 462 mg BID
- flushing (use ASA)
- pretest: CBC w/ diff
- monitor: CBC w/ diff & LFTs q3 mo for 1y; if ALC <0.8, switch

Mods
Fingolimod (Gilenya), Siponimod (Mayzent), Ozanimod (Zeposia), Ponesimod (Ponvory)
- S1P modulator
- once daily
- macular edema, bradycardia
- pretest: CBC w/ diff, LFTs, VZV, ophtho eval (4 mo after starting), derm eval (yearly, r/o skin cancer), pregnancy
- monitor: CBC w/ diff & LFTs 1-3-6, then q3 mo; if ALC <0.2, switch

Cladribine (Mavenclad)
- cytotoxic purine analog
- 1st year: 10 or 20 mg/d for 5d; after 30d, repeat; 2nd year repeat the same scheme
- URI
- pretest: CBC w/diff, HBV/HCV, HIV, LFTs, TB, VZV, pregnancy
- monitor: CBC w/ diff of 2&6 mo after cycle; if ALC <0.5, switch

High
Alemtuzumab (Lemtrada)
- CD52 ab
- 1st year: IV 12 mg/d for 5d (give steroids 3d); 2nd year repeat
- autoimmune thyroid disease
- pretest: CBC w/ diff, HBV&HCV, LFT, TB, TSH, VZV, UA, HPV screen, skin exam (annually)
- monitor: CBC w/ diff, Cr, and UA q1 mo; lymphocyte subset and TSH q3 mo

Natalizumab (Tysabri)
- VLA-4 antibody
- 300 mg IV q1-2 months
- PML, arthralgia
- pretest: CBC w/ diff, JCV, LFT, base MRI
- monitor: JCV & LFT q3 mo
*pregnancy up to 32 wks

PML surveilance
- JCV q3 mo
- if positive: bMRI looking for new lesions, serum natalizumab Ab, LP for JCV in CSF, hold infusion crossing to CD20 (do not hold > 4 weeks risk of rebound)


Negative: <0.2; Indeterminate: 0.2 - <0.4; Positive: >=0.4

Anti-CD20
chemiric (ximab), humanized (zumab), and fully human (umab)
rituximab (rituxan), ublituximab (briumvi), ocrelizumab (ocrevus), ofatumab (kesimpta)
- hypogammaglobulin and red vaccine efficecay
- pretest: CBC w/ diff, BMP, HBV&HCV, immunoglobulins (IgA, G, and M), lymphocyte subset (B&T),  TB, VZV (shringrix vaccine, if negative)
- monitor: all tests before each infusion
* if IgG < 500 give IVIG


Alpha-synuclein tests

Alpha-synuclein tests

Syn-One Test for skin
SynTap Test for CSF

212. Intersection of Autonomic Dysfunction and Parkinson’s Disease: Insights Into Neurogenic and Classical Orthostatic Hypotension

Article type: Narrative Review
Article title: Intersection of Autonomic Dysfunction and Parkinson’s Disease: Insights Into Neurogenic and Classical Orthostatic Hypotension

Journal: Cureus
Year: 2025
Authors: Jamir Pitton Rissardo, Masoumeh Rashidi, Fatemeh Rashidi, Khalil I. Hmedat, Ibrahim Khalil, Hania Moharam, Mallak Bahar, Ali Dway, Omesh Prathiraja, Ana Leticia Fornari Caprara, Maleesha Jayasinghe
E-mail: jamirrissardo@gmail.com

ABSTRACT
Neurogenic orthostatic hypotension (nOH) and classical orthostatic hypotension (OH) are prevalent non-motor manifestations of Parkinson’s disease (PD). They can significantly impact quality of life, increasing the risk of falls, cognitive decline, and functional impairment. Despite the high prevalence and clinical impact of neurogenic orthostatic hypotension and OH in PD, no comprehensive consensus integrates recent advances in pathophysiology, diagnostic tools, and personalized treatment. This review synthesizes current evidence to bridge this gap, offering a practical framework for clinicians to improve patient outcomes. Neurogenic orthostatic hypotension in PD results from complex interactions between central and peripheral autonomic dysfunction, alpha-synuclein accumulation, baroreflex failure, and medication effects. Its prevalence increases with disease progression and age. Clinical evaluation remains the cornerstone of diagnosis, supported by specialized testing such as the active standing test, ambulatory blood pressure monitoring, and autonomic function assessments. Management requires a tailored approach, combining non-pharmacologic strategies, such as fluid and salt intake optimization, compression garments, and physical counter-maneuvers, with pharmacological treatments, including midodrine, droxidopa, and fludrocortisone. Emerging therapies and ongoing clinical trials offer promising avenues for future interventions. Early recognition and individualized management of OH are critical in PD care.
Keywords: autonomic failure; baroreflex dysfunction; dysautonomia; neurogenic orthostatic hypotension; nocturnal hypertension; non-motor symptom; parkinson’s disease; postprandial hypotension; prodromal; supine hypertension.

Full text available at:

DOI

Citation
Pitton Rissardo J, Rashidi M, Rashidi F, Hmedat KI, Khalil I, Moharam H, Bahar M, Dway A, Prathiraja O, Fornari Caprara AL, Jayasinghe M. Intersection of Autonomic Dysfunction and Parkinson’s Disease: Insights Into Neurogenic and Classical Orthostatic Hypotension. Cureus 2025;17: e88487. doi:10.7759/cureus.88487

Table 1. Epidemiological studies on the incidence and prevalence of orthostatic hypotension in Parkinson's disease. BP: blood pressure; OH: orthostatic hypotension; PD Parkinson’s disease; PPMI: Parkinson’s progression markers initiative; nOH: neurogenic orthostatic hypotension; RR: relative risk; IV: intravenous; MIBG: meta-iodobenzylguanidine.

Table 2. Key distinguishing features of neurogenic versus non-neurogenic orthostatic hypotension. ADH: anti-diuretic hormone (vasopressin); ANS: autonomic nervous system; BP: blood pressure; MSA: multiple system atrophy; NE: norepinephrine; PAF: pure autonomic failure; PD: Parkinson’s disease; RAAS: renin-angiotensin-aldosterone system; SBP: systolic blood pressure; OH: orthostatic hypotension.

Table 3. Diagnostic tests to evaluate orthostatic hypotension. ABPM: ambulatory blood pressure monitoring; ANS: autonomic nervous system; BP: blood pressure; ECG: electrocardiogram; HR: heart rate; nOH: neurogenic orthostatic hypotension; OH: orthostatic hypotension; QST: quantitative sudomotor axon reflex test.

Table 4. Management of orthostatic hypotension in Parkinson’s disease. BP: blood pressure; DBP: diastolic blood pressure; FC: fludrocortisone; MBP: mean blood pressure; OH: orthostatic hypotension; PD: Parkinson’s disease; SBP: systolic blood pressure; TID: three times a day; NA: not applicable.

Figure 1. Diagnosis of orthostatic hypotension in Parkinson's disease. OH: orthostatic hypotension; PD: Parkinson's disease; BP: blood pressure; SCOPA-AUT: Scales for Outcomes in Parkinson's disease - Autonomic Dysfunction; COMPASS: Composite Autonomic Symptom Scale; OHQ: Orthostatic Hypotension Questionnaire; OGS: Orthostatic Grading Scale.

Figure 2. Four-phase treatment strategy of orthostatic hypotension in Parkinson's disease. BP: blood pressure.

Figure 3. Complex factors underpinning orthostatic hypotension in Parkinson's disease. OH: orthostatic hypotension.

Watershed Stroke

Watershed Stroke

Deep watershed - c/f hypoperfusion
Cortical watershed - c/f hypoperfusion, but think on emboli

Transient global amnesia (TGA)

Transient global amnesia (TGA)

- patient remembers the thing that was doing just before "I was ****"
- < 24h of symptoms
- classical imaging finding is hippocampal stroke
- related to post-exercise, sexual intercourse, and OUD

CNS Vasculitis

CNS Vasculitis

Primary versus seconday
- primary uncommon systemic signs (normal ESR/CRP)

Features
- only vessel changes in DSA, normal CT and MRI
> MC confusion: ICAD consider risk factors
> MRA overestimates degree of stenosis
*MRA w/o contrast is based on blood flow; MRI w/ contrast is on contrast
- LP = 1/2 inc WBC and 1/2 inc protein
- ongoing headaches
- cortical & subcortical involvement

Stroke - Dysphagia

Dysphagia

SPT
- consult, if fails bedside swallowing
- SPT examination
> cognitive-linguist screen: attention, memory, executive, language
> oral-motor eval: face muscle; lingual and labial muscles strength, rate, and ROM; dentition; cough; voice; speech
> swallow: PO trial w/ ice chips; oral phase (acceptance, mastication); pharyngeal phase (HLE palpation, cough?)

MBSS & FEES

DHT

PEG-tube
- IR, GI, or surgery
- contraindication: stomach remnant absence

JJ tube

TPN
- PICC team for PICC line and consent
- infection risk, especially fungal
- bad if normal intestinal funciton

Phagenyx
- 3 sessions (at bedside) in 3 consecutive days, repeat swallowing study (no need to remove), and can repeat more 3 days
- time-consuming
- patient feels some sensation during sessions, no major complications
- price 5K$
- some rules
> switch the TFs to "no fiber-rich feeds"
> convert meds to liquid/IV; if unable, administer meds crushed one at a time and manually flushed (kangaroo pump does not facilitate enough pressure)
> write in patient's room the depth (55cm)
> If clogged, you can do whatever you need to unclog
> manual flushes as able
> not-MRI compatible
> place bridal

Physician Screening Protocol
- only to give initial meds per oral route
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