Figure 1
Search strategy for the systematic review of cranial functional movement disorders.
Table 1
Demographic and clinical characteristics of the cranial functional movement disorders patients.
| Demographic and clinical characteristics (n = 26) | Findings | |
|---|---|---|
| 1. | AGE | Mean ± standard deviation (SD): 33.96 ± 16.94 (Range: 11 to 83) years |
| Children (age of onset <18 years) | 5 (19.23%) | |
| Adults (18–60 years) | 19 (73.07%) | |
| Elderly (age of onset >60 years) | 2 (7.69%) | |
| 2. | MALE: FEMALE | 10:16 |
| 3. | DURATION | Mean ± SD (402.03 ± 534.97), Range: 1 day–6 years |
| 4. | ONSET | |
| Abrupt: 7 (26.92%) | Gradual: 19 (73.07%) | |
| 5. | LEVEL OF CERTAINTY | |
| Documented: 24 (92.30%) | Clinically established: 2 (7.69%) | |
| 6. | PHENOMENOLOGY | |
| Ocular/peri-ocular: 4 (15.38%) Blepharospasm: 1 (3.8%) Ptosis 1 (3.8%) Eyelid myoclonus: 1 (3.8%) Eyebrow dyskinesia: 1 (3.8%) | Facial: 10 (38.46%) Hemifacial spasm: 4 (15.38%) Orofacial dyskinesia: 5 (19.23%) Pure facial dyskinesia: 1 (3.8%) | |
| Oromandibular: 5 (19.23%) Lingual: 4 (15.38%) | Palatal: 1 (3.8%) Speech: 2 (7.69%) | |
| 7. | EXTRA-CRANIAL MOVEMENTS | 10 (38.46%) |
| Shoulder shrugging: 2 (7.69%) | Twisting movements of upper limbs and neck: 1 (3.8%) | |
| Upper limb tremors only: 3 (11.53%) | Neck dystonia: 1 (3.8%) | |
| Upper limb tremors with lower limb tremors: 1 (3.8%) | Functional hemiparesis with Numbness: 1 (3.8%) | |
| Writing problems: 1 (3.8%) | ||
| 8. | PRECIPITATING FACTORS | |
| Emotional stress: 8 (30.76%) | Road traffic accident: 1 (3.8%) | |
| Financial constraints: 6 (23.07%) | Gastric upset: 1 (3.8%) | |
| Job pressure and work-related stress: 2 (7.69%) | Claustrophobia: 1 (3.8%) | |
| Refusal to go to boarding school: 1 (3.8%) | Keen observation of his own face while shaving: 1 (3.8%) | |
| Brother went missing from home: 1 (3.8%) | ||
| 9. | ASSOCIATED ILLNESSSES | |
| Headache: 4 (15.38%) | ||
| Neuropsychiatric disturbances: 7 (26.92%) Unspecified: (3.8%) Anxiety: 3 (11.53%) Depression: 2 (7.69%) Feelings of loneliness and isolation: 1 (3.8%) | Organic illnesses; 4 (15.38%) Subdural hematoma: 1 (3.8%) Seizure disorder: 1 (3.8%) Organic cervical dystonia: 1 (3.8%) Intracranial mass: 1 (3.8%) | |
| 10. | OUTCOME | |
| Improved: 9 (34.61%) | Did not improve: 4 (15.38%) | |
| Partially improved: 13 (50%) |
Table 2
Clinical characteristics of 26 patients of cranial functional movement disorders.
| Case # | Age (years)/Sex (M: Male, F: Female) | Duration of illness | Onset-abrupt/gradual | Predominant cranial movement disorder | Additional cranial movement disorder | Additional extracranial movement disorder | Precipitating factor | Associated illness | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Case 1 | 34/F | 5 days | Abrupt | Left hemifacial spasm | Jaw deviation to the left side | – | Fight with husband | – | Improved |
| Case 2 | 24/F | 3 months | Gradual | Variable hemifacial spasm | Deviation of mouth to one side | Tremulousness of right upper and lower limbs | Family conflicts | – | Partially improved |
| Case 3 | 16/F | 3 years | Gradual | Right hemifacial spasm | – | – | Family stress | Headache (Migraine) | Partially improved |
| Case 4 | 11/F | 5 months | Gradual | Bilateral ptosis (Right > Left) | – | – | Refusal to go to boarding school | – | Partially improved |
| Case 5 | 24/F | One and half year | Gradual | Chin tremor | Twitching of left angle of mouth | Bilateral postural hand tremors with distractibility | Anxiety regarding her imminent marriage | – | Partially improved |
| Case 6 | 30/F | 3 days | Abrupt | Tongue dystonia | – | – | Death of father-in-law who was the sole earning member of family and financial constraints | – | Improved |
| Case 7 | 16/F | 4 months | Gradual | Palatal tremor | – | – | Brother went missing from home, sick uncle at home | Left occipital ganglioglioma | Partially improved |
| Case 8 | 83/F | 4 months | Gradual | Lip-smacking movements with abnormal noises (orofacial dyskinesias) | – | Bilateral shoulder shrugging | Emotional stress as she was forced to stay away from her son | – | Improved |
| Case 9 | 40/M | 2 days | Abrupt | Stuttering and effortful speech | – | Bilateral hand tremors that are variable, distractibility present | Financial loss due to short-circuit at home | – | Improved |
| Case 10 | 40/F | 1 day | Abrupt | Child-like prosody | – | Left-sided functional hemiparesis with numbness | Financial constraints | Anxiety, depression, headaches | Partially improved |
| Case 11 | 35/M | 6 years | Gradual | Eyebrow dyskinesia | Abnormal tongue movements | Bilateral hand tremors with distractibility | Financial issues | – | Improved |
| Case 12 | 25/M | 3 years | Gradual | Orofacial dyskinesia | – | Twisting movements of upper limb and neck | – | Anxiety, loneliness, avoids friends | Partially improved |
| Case 13 | 62/M | 15 days | Gradual | Orofacial dyskinesia | – | Bilateral shoulder shrugging | – | Subdural hematoma | Partially improved |
| Case 14 | 21/M | One month | Gradual | Orofacial dyskinesia | – | – | – | Neuropsychiatric disturbances | Partially improved |
| Case 15 | 37/M | One year | Gradual | Bruxism | – | – | Job pressure | – | Partially improved |
| Case 16 | 65/F | 2 days | Abrupt | Mouth-opening dystonia | – | – | Claustrophobia during MRI | Organic cervical dystonia | Improved |
| Case 17 | 31/M | 5 months | Gradual | Tongue tremor | – | – | Looking at himself in the mirror while shaving | – | Improved |
| Case 18 | 41/F | One year | Gradual | Oromandibular dyskinesia | Jaw deviation to the left side | – | Husband stays away in Dubai | – | Partially improved |
| Case 19 | 48/M | 2–1/2 years | Gradual | Blepharospasm | Ocular tics for 1 year | Writing problems for 10 days | Work-related pressure | Anxiety | Partially improved |
| Case 20 | 25/F | 1 day | Abrupt | Tongue dystonia | – | – | Gastric upset | – | Improved |
| Case 21 | 50/F | 5 months | Gradual | Right hemifacial spasm | – | – | Depression | Not improved | |
| Case 22 | 40/M | 4 months | Gradual | Facial dyskinesia | – | – | Financial constraints | – | Not improved |
| Case 23 | 20/F | 4 days | Abrupt | Jaw spasm | – | – | Road traffic accident 4 days back | – | Not improved |
| Case 24 | 13/M | 3 years | Gradual | Bilateral eyelid myoclonus | – | – | Fight with a friend | Seizure disorder for 5 years, headache for 3 days | Partially improved |
| Case 25 | 16/F | 3 years | Gradual | Tongue protrusion | – | Functional neck dystonia | Financial constraints | – | Improved |
| Case 26 | 36/F | 2 years | Gradual | Orofacial dyskinesia | – | – | Fight with husband | Headache | Not improved |
Video
Cranial functional movement disorder patients (cases 1–9): Abnormal movements, variability and distractibility are seen in different patients.
Case 1: A 34-year old lady with an abrupt onset fixed left-hemifacial spasm of 5 days duration after a squabble with her husband.
Case 2: A 25-year old female with gradual onset variable hemifacial spasm of either side of her face for the last 3 months with bilateral tremulousness of her upper and lower limbs as well. She cited family conflicts as a stress factor.
Case 3: A 16-year old female with gradual onset hemifacial spasm of either side of her face for the last 3 years which is variable and distractible while finger tapping and when her face is being touched by a pen.
Case 4: An 11-year old girl presented with bilateral (right > left) intermittent ptosis for 5 months that started to develop after she was being forced to go to the boarding school. On finger tapping with the right hand, there is entrainment in the eyelid blinking.
Case 5: A 24-year old female has chin tremors of one-and-a-half-year duration. She reported anxiety regarding her impending marriage. On finger tapping from the left hand, her chin tremor decreases significantly.
Case 6: A 25-year old female developed abrupt onset abnormal movements of the tongue for 3 days. She had significant difficulty in speaking (Segment 1). Her symptoms improved significantly within a week following counselling (Segment 2).
Case 7: A 16-year girl who presented with a palatal tremor of 4 months duration that was variable and could be completely suppressed on manually depressing the tongue with a spatula.
Case 8: A 83-year old woman developed 2 episodes of abnormal lip-smacking movements with bizarre noises lasting for about 5 days each for 4 months that was precipitated due to emotional stress (Segment 1). Her symptoms used to subside while engaged in conversation (Segment 2).
Case 9: A 40-year old male with abrupt onset stuttering of speech of 2 days duration. On examination, bilateral hand tremor is also present which is variable and distracted while finger tapping from the left hand.