Introduction: Benign adult familial myoclonus epilepsy (BAFME) is an autosomal dominant disorder characterized by adult-onset cortical tremor and infrequent generalized seizures. Treatment options for managing involuntary movements in BAFME remain limited. Case Presentation: Here, we present 2 cases involving individuals with BAFME who underwent Vim thalamotomy for tremor-like myoclonus. Despite the intervention, neither patient experienced any improvement in their symptoms. Conclusion: These findings suggest that the Vim may not contribute to the pathophysiology of tremor-like myoclonus in BAFME, despite the established efficacy of Vim thalamotomy in the treatment of essential tremor.

1.
Berkovic
SF
,
Striano
P
,
Tsuji
S
.
History of familial adult myoclonus epilepsy/benign adult familial myoclonic epilepsy around the world
.
Epilepsia
.
2023
;
64
(
Suppl 1
):
S3
8
.
2.
Peters
L
,
Depienne
C
,
Klebe
S
.
Familial adult myoclonic epilepsy (FAME): clinical features, molecular characteristics, pathophysiological aspects and diagnostic work-up
.
Med Genet
.
2021
;
33
(
4
):
311
8
.
3.
Wang
G
,
Song
Y
,
Su
J
,
Fan
Z
,
Xu
L
,
Fang
P
, et al
.
Altered cerebellar-motor loop in benign adult familial myoclonic epilepsy type 1: the structural basis of cortical tremor
.
Epilepsia
.
2022
;
63
(
12
):
3192
203
.
4.
Schuurman
PR
,
Bosch
DA
,
Bossuyt
PM
,
Bonsel
GJ
,
van Someren
EJ
,
de Bie
RM
, et al
.
A comparison of continuous thalamic stimulation and thalamotomy for suppression of severe tremor
.
N Engl J Med
.
2000
;
342
(
7
):
461
8
.
5.
Horisawa
S
,
Nonaka
T
,
Kohara
K
,
Mochizuki
T
,
Kawamata
T
,
Taira
T
.
Bilateral radiofrequency ventral intermediate thalamotomy for essential tremor
.
Stereotact Funct Neurosurg
.
2023
;
101
(
1
):
30
40
.
6.
Dallapiazza
RF
,
Lee
DJ
,
De Vloo
P
,
Fomenko
A
,
Hamani
C
,
Hodaie
M
, et al
.
Outcomes from stereotactic surgery for essential tremor
.
J Neurol Neurosurg Psychiatry
.
2019
;
90
(
4
):
474
82
.
7.
Kuncel
AM
,
Turner
DA
,
Ozelius
LJ
,
Greene
PE
,
Grill
WM
,
Stacy
MA
.
Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome
.
Clin Neurol Neurosurg
.
2009
;
111
(
3
):
303
6
.
8.
Wang
JW
,
Li
JP
,
Wang
YP
,
Zhang
XH
,
Zhang
YQ
.
Deep brain stimulation for myoclonus-dystonia syndrome with double mutations in DYT1 and DYT11
.
Sci Rep
.
2017
;
7
:
41042
.
9.
Asahi
T
,
Kashiwazaki
D
,
Dougu
N
,
Oyama
G
,
Takashima
S
,
Tanaka
K
, et al
.
Alleviation of myoclonus after bilateral pallidal deep brain stimulation for Lance-Adams syndrome
.
J Neurol
.
2015
;
262
(
6
):
1581
3
.
10.
Ramdhani
RA
,
Frucht
SJ
,
Kopell
BH
.
Improvement of post-hypoxic myoclonus with bilateral pallidal deep brain stimulation: a case report and review of the literature
.
Tremor Other Hyperkinet Mov
.
2017
;
7
:
461
.
11.
Kobayashi
K
,
Katayama
Y
,
Otaka
T
,
Obuchi
T
,
Kano
T
,
Nagaoka
T
, et al
.
Thalamic deep brain stimulation for the treatment of action myoclonus caused by perinatal anoxia
.
Stereotact Funct Neurosurg
.
2010
;
88
(
4
):
259
63
.
12.
Wille
C
,
Steinhoff
BJ
,
Altenmüller
DM
,
Staack
AM
,
Bilic
S
,
Nikkhah
G
, et al
.
Chronic high-frequency deep-brain stimulation in progressive myoclonic epilepsy in adulthood: report of five cases
.
Epilepsia
.
2011
;
52
(
3
):
489
96
.
13.
Di Giacopo
A
,
Baumann
CR
,
Kurthen
M
,
Capecchi
F
,
Sürücü
O
,
Imbach
LL
.
Selective deep brain stimulation in the substantia nigra reduces myoclonus in progressive myoclonic epilepsy: a novel observation and short review of the literature
.
Epileptic Disord
.
2019
;
21
(
3
):
283
8
.
14.
Anderson
DG
,
Németh
AH
,
Fawcett
KA
,
Sims
D
,
Miller
J
,
Krause
A
.
Deep brain stimulation in three related cases of North Sea progressive myoclonic epilepsy from South Africa
.
Mov Disord Clin Pract
.
2017
;
4
(
2
):
249
53
.
15.
Horisawa
S
,
Miyao
S
,
Hori
T
,
Kohara
K
,
Kawamata
T
,
Taira
T
.
Comorbid seizure reduction after pallidothalamic tractotomy for movement disorders: revival of Jinnai’s Forel-H-tomy
.
Epilepsia Open
.
2021
;
6
(
1
):
225
9
.
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